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Knoedler L, Hoch CC, Baecher H, Geldner B, Niederegger T, Knoedler S, Lellouch AG, Heiland M, Koerdt S, Alfertshofer M. Therapeutic Outcomes in 691 Angiosarcomas of the Head and Neck-A Retrospective SEER Study. J Craniofac Surg 2024:00001665-990000000-02220. [PMID: 39607328 DOI: 10.1097/scs.0000000000010937] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2024] [Accepted: 11/08/2024] [Indexed: 11/29/2024] Open
Abstract
Angiosarcomas (AS) are an aggressive subtype of head and neck tumors. Current outcome research is limited to single-surgeon and/or single-institution data. This study analyzes the Surveillance, Epidemiology, and End Results program (SEER) database data to improve outcomes of head and neck AS. In this study, the authors accessed the SEER database from 2000 to 2020 to identify patients who underwent surgical and/or nonsurgical treatment of head and neck AS. Data included demographics, diagnostic workup, tumor characteristics, treatment pathways, and therapy outcomes. The study included 691 patients (210 females, 481 males) with AS of the head and neck. The median tumor size was 40 mm. Most patients (63%) underwent surgery, with surgery followed by radiation being the most common treatment combination (50%). Median survival was 16 months. Patients treated with surgery showed significantly longer survival (18 mo) compared with nonsurgery cases (12 mo, P<0.001). Neoadjuvant treatment significantly improved survival (28 mo) compared with non-neoadjuvant cases (15 mo, P=0.023). Adjuvant treatment also improved overall survival (19 mo) versus nonadjuvant treatment (14 mo, P<0.001). There was no significant difference between neoadjuvant and adjuvant treatment outcomes (P=0.270). Bottom of Form This study compares the outcomes of multimodal treatments, especially surgery and radiotherapy, for head and neck AS, emphasizing tailored approaches to optimize patient outcomes and extend survival. Future research should refine these strategies.
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Affiliation(s)
- Leonard Knoedler
- Department of Oral and Maxillofacial Surgery, Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin
| | - Cosima C Hoch
- Department of Otolaryngology, Head and Neck Surgery, TUM School of Medicine and Health, Technical University of Munich, Munich
| | - Helena Baecher
- Department of Oral and Maxillofacial Surgery, University Hospital Regensburg, Regensburg, Germany
| | - Benedikt Geldner
- Department of Oral and Maxillofacial Surgery, University Hospital Regensburg, Regensburg, Germany
| | - Tobias Niederegger
- Department of Oral and Maxillofacial Surgery, University Hospital Regensburg, Regensburg, Germany
| | - Samuel Knoedler
- Vascularized Composite Allotransplantation Laboratory, Massachusetts General Hospital, Harvard Medical School
| | - Alexandre G Lellouch
- Vascularized Composite Allotransplantation Laboratory, Massachusetts General Hospital, Harvard Medical School
- Shriners Children's Boston, Boston, MA
- Cedars-Sinai Medical Center, Los Angeles, CA
| | - Max Heiland
- Department of Oral and Maxillofacial Surgery, Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin
| | - Steffen Koerdt
- Department of Oral and Maxillofacial Surgery, Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin
| | - Michael Alfertshofer
- Department of Oral and Maxillofacial Surgery, Charité - Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin
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Catalo M, Afonso M, Verdelho A, Aguiar A, Arantes M. Extramedullary Intradural Primary Spinal Angiosarcoma: A Case Study. Cureus 2024; 16:e74767. [PMID: 39734979 PMCID: PMC11682733 DOI: 10.7759/cureus.74767] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/29/2024] [Indexed: 12/31/2024] Open
Abstract
Angiosarcoma is a rare soft tissue sarcoma, namely when it presents as a primary intradural extramedullary spinal neoplasm, with only one case of non-vertebral origin reported in the literature. We present the case of a 51-year-old woman with neurological symptoms of paraparesis and constipation who underwent a magnetic resonance imaging (MRI) that revealed a well-demarcated, predominantly homogeneous, intensely enhancing intradural extramedullary lesion in T2-weighted images. Histology, immunohistochemistry, and genetics of the lesion showed an angiosarcoma. The patient received adjuvant radiation therapy (RT) and is currently stable, showing no signs of recurrence, in the third year of follow-up. Our work demonstrates a rare case of extramedullary intradural primary spinal angiosarcoma, highlighting the challenges associated with its diagnosis. The multidisciplinary approach, including surgery and adjuvant RT, has been shown to effectively control the disease. Although the prognosis is generally poor, the presented case shows apparent success.
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Affiliation(s)
- Miguel Catalo
- Radiation Oncology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT
| | - Mariana Afonso
- Pathology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT
| | - António Verdelho
- Neurosurgery, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT
| | - Artur Aguiar
- Radiation Oncology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT
| | - Mavilde Arantes
- Neuroradiology, Instituto Português de Oncologia do Porto Francisco Gentil, Porto, PRT
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Huang J, Liao Z, Hu Y, Cheng Y, Zhong J. Prognostic factors of cutaneous soft tissue sarcomas in children: a SEER population-based study. Arch Dermatol Res 2024; 316:700. [PMID: 39424676 DOI: 10.1007/s00403-024-03450-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/07/2024] [Revised: 09/23/2024] [Accepted: 10/08/2024] [Indexed: 10/21/2024]
Abstract
This study aims to analyze the clinicopathological characteristics and survival outcomes of cutaneous soft tissue sarcomas (CSTS) in children. We selected pediatric cases of CSTS diagnosed between 2000 and 2019 from the Surveillance, Epidemiology, and End Results (SEER) database. Survival rates were calculated using Kaplan-Meier methods. We performed univariate analyses with the log-rank test and multivariate survival analyses using Cox proportional-hazards models to determine factors affecting overall survival (OS). Additionally, we constructed a predictive nomogram based on the outcomes of the Cox regression. A total of 148 pediatric patients with CSTS were reviewed. The median age at diagnosis was 13 years (range: 0-18 years). Prognostically, tumors located on the extremities showed better outcomes compared to those on the head, neck, or trunk. Among the histological types, angiosarcoma had the lowest five-year survival rate at 51.3%, which was substantially lower compared to fibrous histiocytoma and leiomyosarcoma. Cox regression analysis highlighted surgical intervention as the only significant independent prognostic factor for OS, with an increased risk of mortality observed in patients not undergoing surgery. Additionally, patients with distant-stage disease exhibited significantly lower survival rates than those with localized conditions. Pediatric CSTS represents a diverse and infrequent group of tumors, predominantly fibrous histiocytoma and leiomyosarcoma. Surgery was identified as the crucial determinant of survival, underscoring its role in effectively managing these patients.
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Affiliation(s)
- Jian Huang
- Department of Pediatrics, The Second Affiliated Hospital of Anhui Medical University, No. 678 Furong Road, Hefei, Anhui, 230601, China
| | - Zhenqi Liao
- Department of Cardiology, Ganxian Renmin Hospital, Ganzhou, Jiangxi, China
| | - Yilan Hu
- Department of Rehabilitation, Hangzhou First People's Hospita Xiasha Campus, Hangzhou Rehabilitation Hospital, Hangzhou, Zhejiang, China
| | - Yan Cheng
- Department of Pediatrics, The Second Affiliated Hospital of Anhui Medical University, No. 678 Furong Road, Hefei, Anhui, 230601, China.
| | - Jiawang Zhong
- Department of Stomatology, Anji County People's Hospital, Huzhou, Zhejiang, 313300, China.
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Roohani S, Rotermund T, Ehret F, Dziodzio T, Jarosch A, Schäfer FM, Flörcken A, Wittenberg S, Zips D, Kaul D. Angiosarcoma: clinical outcomes and prognostic factors, a single-center analysis. J Cancer Res Clin Oncol 2024; 150:326. [PMID: 38914779 PMCID: PMC11196347 DOI: 10.1007/s00432-024-05835-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2024] [Accepted: 06/04/2024] [Indexed: 06/26/2024]
Abstract
PURPOSE This study sought to investigate oncological outcomes and prognostic factors for patients with angiosarcomas (AS). METHODS This single-center, retrospective cohort study, analyzed histopathologically confirmed AS cases. Primarily diagnosed, locally recurrent and metastatic AS were included. Overall survival (OS), local control (LC) and local progression-free survival (LPFS) were assessed by Kaplan-Meier estimator. Multivariable Cox regression analysis was performed to detect factors associated with OS and LPFS. RESULTS In total, 118 patients with a median follow-up of 6.6 months were included. The majority presented with localized disease (62.7%), followed by metastatic (31.4%) and locally recurrent (5.9%) disease. Seventy-four patients (62.7%) received surgery, of which 29 (39.2%) were treated with surgery only, 38 (51.4%) with surgery and perioperative radiotherapy or chemotherapy, and 7 (9.4%) with surgery, perioperative radiotherapy and chemotherapy. Multivariable Cox regression of OS showed a significant association with age per year (hazard ratio (HR): 1.03, p = 0.044) and metastatic disease at presentation (hazard ratio: 3.24, p = 0.015). For LPFS, age per year (HR: 1.04, p = 0.008), locally recurrent disease at presentation (HR: 5.32, p = 0.013), and metastatic disease at presentation (HR: 4.06, p = 0.009) had significant associations. Tumor size, epithelioid components, margin status, and perioperative RT and/or CTX were not significantly associated with OS or LPFS. CONCLUSION Older age and metastatic disease at initial presentation status were negatively associated with OS and LPFS. Innovative and collaborative effort is warranted to overcome the epidemiologic challenges of AS by collecting multi-institutional datasets, characterizing AS molecularly and identifying new perioperative therapies to improve patient outcomes.
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Affiliation(s)
- Siyer Roohani
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.
- BIH Charité (Junior) Clinician Scientist Program, Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, Charitéplatz 1, 10117, Berlin, Germany.
- Partner site Berlin, a partnership between DKFZ and Charité, German Cancer Consortium (DKTK), Universitätsmedizin Berlin, Berlin, Germany.
| | - Titus Rotermund
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
| | - Felix Ehret
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
- Partner site Berlin, a partnership between DKFZ and Charité, German Cancer Consortium (DKTK), Universitätsmedizin Berlin, Berlin, Germany
| | - Tomasz Dziodzio
- BIH Charité (Junior) Clinician Scientist Program, Berlin Institute of Health at Charité - Universitätsmedizin Berlin, BIH Biomedical Innovation Academy, Charitéplatz 1, 10117, Berlin, Germany
- Department of Surgery, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
| | - Armin Jarosch
- Institute of Pathology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117, Berlin, Germany
| | - Frederik Maximilian Schäfer
- Department of Radiology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität Zu Berlin, Berlin, Germany
| | - Anne Flörcken
- Partner site Berlin, a partnership between DKFZ and Charité, German Cancer Consortium (DKTK), Universitätsmedizin Berlin, Berlin, Germany
- Department of Hematology, Oncology and Tumor Immunology, Charité - Universitätsmedizin Berlin, Corporate member of Freie Universität Berlin and Humboldt, Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
| | - Silvan Wittenberg
- Center for Musculoskeletal Surgery, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Campus Virchow-Klinikum, Augustenburger Platz 1, 13353, Berlin, Germany
| | - Daniel Zips
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
- Partner site Berlin, a partnership between DKFZ and Charité, German Cancer Consortium (DKTK), Universitätsmedizin Berlin, Berlin, Germany
| | - David Kaul
- Department of Radiation Oncology, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Augustenburger Platz 1, 13353, Berlin, Germany
- Partner site Berlin, a partnership between DKFZ and Charité, German Cancer Consortium (DKTK), Universitätsmedizin Berlin, Berlin, Germany
- Health and Medical University Potsdam, Olympischer Weg 1, 14471, Potsdam, Germany
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Iacomino E, Fratini C, Sollima L, Eibenstein A, Barbato C, de Vincentiis M, Minni A, Zoccali F. Surgical Resection and Immediate Reconstruction with a Bilayer Wound Collagen Matrix of a Rare Oral Angiosarcoma: A Case Report. Diseases 2024; 12:117. [PMID: 38920549 PMCID: PMC11202842 DOI: 10.3390/diseases12060117] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/22/2024] [Revised: 05/21/2024] [Accepted: 05/23/2024] [Indexed: 06/27/2024] Open
Abstract
Angiosarcomas are malignant vascular tumors that commonly occur on the skin of the head and neck, breast, or scalp. Oral angiosarcoma is a rare tumor (0.0077% of all cancers in Europe), and regarding this atypical localization, no formal treatment trials have been conducted yet. We present a case of a 58-year-old female patient with a diagnosis of oral angiosarcoma. After tumor excision was performed by transoral surgical approach, immediate reconstruction of the intraoral surgical defects was made using Integra® bilayer wound collagen matrix. A skin regeneration technique has previously been reported to provide good healing for defects of buccal resection, preventing postoperative cicatricial fibrosis.
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Affiliation(s)
- Enzo Iacomino
- Department of Life, Health, and Environmental Sciences, Università degli Studi di L’Aquila, 67100 L’Aquila, Italy;
| | - Chiara Fratini
- Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy; (C.F.); (M.d.V.); (F.Z.)
| | - Laura Sollima
- Anatomy and Pathological Histology Division, ASL 1 Avezzano-Sulmona-L’Aquila, San Salvatore Hospital, 67100 L’Aquila, Italy;
| | - Alberto Eibenstein
- Department of Applied Clinical and Biotechnological Sciences, University of Aquila, 67100 L’Aquila, Italy;
| | - Christian Barbato
- Institute of Biochemistry and Cell Biology (IBBC-CNR), Department of Sense Organs, Sapienza University Rome, Policlinico Umberto I, 00161 Roma, Italy
| | - Marco de Vincentiis
- Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy; (C.F.); (M.d.V.); (F.Z.)
| | - Antonio Minni
- Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy; (C.F.); (M.d.V.); (F.Z.)
- Division of Otolaryngology-Head and Neck Surgery, ASL Rieti-Sapienza University, Ospedale San Camillo de Lellis, 02100 Rieti, Italy
| | - Federica Zoccali
- Department of Sense Organs, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy; (C.F.); (M.d.V.); (F.Z.)
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6
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Holm CE, Ørholt M, Talman ML, Abebe K, Thorn A, Baad-Hansen T, Petersen MM. A Population-Based Long-Term Follow-Up of Soft Tissue Angiosarcomas: Characteristics, Treatment Outcomes, and Prognostic Factors. Cancers (Basel) 2024; 16:1834. [PMID: 38791913 PMCID: PMC11120488 DOI: 10.3390/cancers16101834] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/28/2024] [Revised: 05/06/2024] [Accepted: 05/10/2024] [Indexed: 05/26/2024] Open
Abstract
Angiosarcoma is a rare aggressive and understudied soft tissue sarcoma with pending evidence-based treatment guidelines due to varying study cohorts and inconsistent outcome measures. Surgery with wide resection is currently considered to be the cornerstone in management. In a population-based cohort identified from Danish National Health Registers between 2000 and 2017, this study aimed to define prognostic factors in patients with newly diagnosed soft tissue angiosarcoma. Kaplan-Meier survival analysis demonstrated 5-year overall survival of 28%. Competing risk analysis demonstrated cumulative incidence of local recurrence of 30% and metastasis of 43%. Multivariable Cox models among 154 included patients demonstrated age above 60 years and metastasis to be independently associated with worse overall survival. Cutaneous tumors, surgery, and negative resection margin were independently associated with improved overall survival. Adjuvant oncological treatment did not improve overall survival, risk of metastasis, or recurrence. Negative margin was not associated with lower risk of recurrence and metastasis. We conclude that, despite demonstrated improved survival after surgery with wide resection, overall survival remains poor.
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Affiliation(s)
- Christina Enciso Holm
- Department of Orthopedic Surgery, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark; (A.T.); (M.M.P.)
| | - Mathias Ørholt
- Department of Plastic Surgery and Burns Treatment, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark; (M.Ø.); (K.A.)
| | - Maj-Lis Talman
- Department of Pathology, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark;
| | - Kiya Abebe
- Department of Plastic Surgery and Burns Treatment, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark; (M.Ø.); (K.A.)
| | - Andrea Thorn
- Department of Orthopedic Surgery, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark; (A.T.); (M.M.P.)
| | - Thomas Baad-Hansen
- Department of Orthopaedic Surgery, Tumor Section, Aarhus University Hospital, Palle Juul-Jensen Blvd, 8200 Aarhus, Denmark;
| | - Michael Mørk Petersen
- Department of Orthopedic Surgery, Rigshospitalet, University of Copenhagen, Blegdamsvej 3, 2100 Copenhagen, Denmark; (A.T.); (M.M.P.)
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7
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Chang HY, Dermawan JK, Kuba MG, Crago AM, Singer S, Tap W, Chi P, D’Angelo S, Rosenbaum E, Antonescu CR. Clinicopathologic and molecular correlates to neoadjuvant chemotherapy-induced pathologic response in breast angiosarcoma. Genes Chromosomes Cancer 2024; 63:e23240. [PMID: 38722225 PMCID: PMC11740977 DOI: 10.1002/gcc.23240] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2024] [Revised: 03/31/2024] [Accepted: 04/08/2024] [Indexed: 06/02/2024] Open
Abstract
Both primary and secondary breast angiosarcoma (AS) are characterized by multifocal presentation and aggressive behavior. Despite multimodality therapy, local and distant relapse rates remain high. Therefore, neoadjuvant chemotherapy (NACT) is employed to improve the R0 resection rates and survival, but its benefits remain controversial. Herein, we investigate pathologic and molecular correlates to NACT-induced histologic response in a group of 29 breast AS, 4 primary and 25 radiation-associated (RA). The two NACT regimens applied were anthracycline- and non-anthracycline-based. The pathologic response grade was defined as: I: ≤ 50%, II: 51%-90%, III: 91%-99%, and IV: 100%. An additional 45 primary AS and 102 RA-AS treated by surgery alone were included for survival comparison. The genomic landscape was analyzed in a subset of cases and compared to a cohort of AS without NACT on a paired tumor-normal targeted DNA NGS platform. All patients were females, with a median age of 31 years in primary AS and 68 years in RA-AS. All surgical margins were negative in NACT group. The NACT response was evenly divided between poor (Grades I-II; n = 15) and good responders (Grades III-IV; n = 14). Mitotic count >10/mm2 was the only factor inversely associated with pathologic response. By targeted NGS, all 10 post-NACT RA-AS demonstrated MYC amplification, while both primary AS harbored KDR mutations. TMB or other genomic alterations did not correlate with pathologic response. All four patients with Grade IV response remained free of disease. The good responders had a significantly better disease-specific survival (p = 0.04). There was no survival difference with NACT status or the NACT regimens applied. However, NACT patients with MYC-amplified tumors showed better disease-free survival (p = 0.04) compared to MYC-amplified patients without NACT. The overall survival of NACT group correlated with size >10 cm (p = 0.02), pathologic response (p = 0.04), and multifocality (p = 0.01) by univariate, while only size >10 cm (p = 0.03) remained significant by multivariate analysis.
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Affiliation(s)
- Hsin-Yi Chang
- Department of Pathology and Laboratory Medicine, Taipei Veterans General Hospital, Taipei, Taiwan
- Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - Josephine K. Dermawan
- Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Maria Gabriela Kuba
- Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - Aimee M. Crago
- Department of Surgery, Gastric Mixed Tumor Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
- Department of Surgery, Weill Cornell Medical Center, New York, New York, USA
| | - Samuel Singer
- Department of Surgery, Gastric Mixed Tumor Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
| | - William Tap
- Department of Medicine, Sarcoma Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
- Department of Medicine, Weill Cornell Medical Center, New York, New York, USA
| | - Ping Chi
- Department of Medicine, Sarcoma Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
- Department of Medicine, Weill Cornell Medical Center, New York, New York, USA
| | - Sandra D’Angelo
- Department of Medicine, Sarcoma Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
- Department of Medicine, Weill Cornell Medical Center, New York, New York, USA
| | - Evan Rosenbaum
- Department of Medicine, Sarcoma Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA
- Department of Medicine, Weill Cornell Medical Center, New York, New York, USA
| | - Cristina R. Antonescu
- Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center, New York, New York, USA
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8
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Wang X, Yik-Lok Chung C, Yoshioka A, Hashimoto S, Jimbo H, Tanizawa H, Ohta S, Fukumoto T, Noma KI. Chemo-Senolytic Therapeutic Potential against Angiosarcoma. J Invest Dermatol 2024:S0022-202X(24)00268-9. [PMID: 38570028 DOI: 10.1016/j.jid.2024.03.026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/15/2023] [Revised: 03/02/2024] [Accepted: 03/20/2024] [Indexed: 04/05/2024]
Abstract
Angiosarcoma is an aggressive soft-tissue sarcoma with a poor prognosis. Chemotherapy for this cancer typically employs paclitaxel, a taxane (genotoxic drug), although it has a limited effect owing to chemoresistance to prolonged treatment. In this study, we examine an alternative angiosarcoma treatment approach that combines chemotherapeutic and senolytic agents. We find that the chemotherapeutic drugs cisplatin and paclitaxel efficiently induce senescence in angiosarcoma cells. Subsequent treatment with the senolytic agent ABT-263 eliminates senescent cells by activating the apoptotic pathway. In addition, expression analysis indicates that senescence-associated secretory phenotype genes are activated in senescent angiosarcoma cells and that ABT-263 treatment downregulates IFN-I pathway genes in senescent cells. Moreover, we show that cisplatin treatment alone requires high doses to remove angiosarcoma cells. In contrast, lower doses of cisplatin are sufficient to induce senescence, followed by the elimination of senescent cells by the senolytic treatment. This study sheds light on a potential therapeutic strategy against angiosarcoma by combining a relatively low dose of cisplatin with the ABT-263 senolytic agent, which can help ease the deleterious side effects of chemotherapy.
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Affiliation(s)
- Xuebing Wang
- Division of Genome Biology, Institute for Genetic Medicine, Hokkaido University, Sapporo, Japan
| | - Claire Yik-Lok Chung
- Division of Genome Biology, Institute for Genetic Medicine, Hokkaido University, Sapporo, Japan
| | - Ai Yoshioka
- Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Shinya Hashimoto
- Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Haruki Jimbo
- Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Hideki Tanizawa
- Division of Genome Biology, Institute for Genetic Medicine, Hokkaido University, Sapporo, Japan
| | - Shinya Ohta
- Division of Genome Biology, Institute for Genetic Medicine, Hokkaido University, Sapporo, Japan
| | - Takeshi Fukumoto
- Division of Dermatology, Department of Internal Related, Kobe University Graduate School of Medicine, Kobe, Japan
| | - Ken-Ichi Noma
- Division of Genome Biology, Institute for Genetic Medicine, Hokkaido University, Sapporo, Japan; Institute of Molecular Biology, University of Oregon, Eugene, Oregon, USA.
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9
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Song Y, Li R, Wang L, Wang H. Case Report: A Rare Case of Primary Angiosarcoma of the Cervix with a Literature Review. Int J Womens Health 2024; 16:265-271. [PMID: 38370343 PMCID: PMC10871135 DOI: 10.2147/ijwh.s439583] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/11/2023] [Accepted: 01/22/2024] [Indexed: 02/20/2024] Open
Abstract
Primary angiosarcomas are a rare type of soft-tissue sarcomas that originate from endothelial cells. These sarcomas can develop in any part of the body and have a poor prognosis. However, they are commonly found in the skin of elderly white men, particularly on the scalp and head region. Primary angiosarcoma of the cervix is exceptionally rare. To date, only two cases of this disease have been reported worldwide. The diagnosis of the disease is difficult microscopically, requiring immunohistochemistry and genetic testing to distinguish. We report a recent case, in which the lesion was preoperatively considered a high-grade endometrial stromal sarcoma. A 35-year-old woman presented with vaginal bleeding and cervical erosions. A high-grade endometrial stromal sarcoma involving the cervix was considered and a modified radical hysterectomy was performed with bilateral salpingo-oophorectomy and sentinel lymph nodes resection. The gene diagnosis performed by fluorescence in situ hybridization for YWHAE translocation fusion was negative excluding a YWHAE-translocated high-grade endometrial stromal sarcoma. A primary angiosarcoma of the cervix was finally diagnosed. Primary angiosarcoma of the cervix is rare, and gynecologic pathologists do not know it well, so it is easy to be wrongly considered. Immunohistochemistry and genetic testing help confirm the diagnosis.
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Affiliation(s)
- Yuelin Song
- Department of Obstetrics and Gynecology, West China Second University Hospital of Sichuan University, Chengdu, People’s Republic of China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, People’s Republic of China
| | - Ruizhe Li
- Department of Obstetrics and Gynecology, West China Second University Hospital of Sichuan University, Chengdu, People’s Republic of China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, People’s Republic of China
| | - Lifei Wang
- Department of Obstetrics and Gynecology, The Fourth Affiliated Hospital Zhejiang University School of Medicine, Yiwu, People’s Republic of China
| | - Hongjing Wang
- Department of Obstetrics and Gynecology, West China Second University Hospital of Sichuan University, Chengdu, People’s Republic of China
- Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu, People’s Republic of China
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10
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Herieka M, Amin K, Kosutic D. An Unexpected Late Recurrence of Breast Irradiation-Induced Angiosarcoma Following Autologous Microvascular Breast Reconstruction. Cureus 2024; 16:e54741. [PMID: 38524062 PMCID: PMC10960949 DOI: 10.7759/cureus.54741] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 02/21/2024] [Indexed: 03/26/2024] Open
Abstract
The authors present the case of a 68-year-old female who developed recurrent angiosarcoma, a rare but recognized complication after breast irradiation therapy in the treatment of breast cancer. Microvascular breast reconstruction was performed after the completion of 10 years of disease-free clinical surveillance. Abdominal tissue was harvested and transferred onto the chest wall with restoration of its blood supply using microsurgical techniques to recreate the breast. Unexpectedly, local recurrence of irradiation-induced angiosarcoma was confirmed in the reconstructed breast 12 years later, a unique finding, given the long latent period and recruitment of tissues from a distant site. It is vital to consider the potential of late recurrence before embarking on complex reconstructions, and this should be discussed with patients who have a history of angiosarcoma. This further emphasizes the importance of long-term surveillance in such a rare, yet aggressive tumor at specialist centers.
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Affiliation(s)
- Mohammed Herieka
- Plastic and Reconstructive Surgery, The Christie NHS Foundation Trust, Manchester, GBR
| | - Kavit Amin
- Plastic and Reconstructive Surgery, The Christie NHS Foundation Trust, Manchester, GBR
| | - Damir Kosutic
- Plastic and Reconstructive Surgery, The Christie NHS Foundation Trust, Manchester, GBR
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11
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Al Laham O, Sharaf Aldeen R, Ibrahim Basha Z, Ali A, Alhanwt A. Dual unifocal primary Epithelioid Angiosarcoma: A case report and review of the literature unveiling a rare genre of neoplasia. Int J Surg Case Rep 2024; 115:109310. [PMID: 38281378 PMCID: PMC10839256 DOI: 10.1016/j.ijscr.2024.109310] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/27/2023] [Revised: 01/24/2024] [Accepted: 01/24/2024] [Indexed: 01/30/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Angiosarcomas are an exceedingly rare and malignant form of soft tissue sarcoma that are derived from endothelial cells. Overall, they comprise <1 % of the total number of soft tissue sarcomas. Due to nonspecific and misleading symptoms, the subsequent clinical presentations can easily result in misdiagnosis. This leads to life-threatening complications for patients. Contemplating this tumor as a differential diagnosis during the preoperative phase allows for essential time-sensitive therapeutic interventions to be accomplished. CASE PRESENTATION Herein, we present the seldom precedented case of a 66-year-old Middle Eastern male who came to our surgical clinic chiefly complaining of an exacerbation of chronic left hypochondriac pain accompanied by gradual inexplicable abdominal distention. Our diagnostic radiological evaluation demonstrated two isolated abdominal mass formations. CLINICAL DISCUSSION Sheer excision of the neoplastic masses with safety margins was successfully executed via open surgery. The stemming histopathological examination through Hematoxylin and Eosin and immunohistochemical staining established the definitive diagnosis of an Epithelioid Angiosarcoma. CONCLUSION Epithelioid Angiosarcomas belong to the category of profoundly rare tumors. The available published literature conveys this rarity through the scarcity of epidemiological parameters and studies. It necessitates being borne in mind when facing similar clinical scenarios so that apt therapeutic interventions can be achieved. Structured diagnostic methods, timely surgical interventions and proper techniques, and comprehensive follow-up patient surveillance protocols are, therefore, merited. After thorough review of the published literature, we reckon herewith that ours is the first documented case from our country of an Epithelioid Angiosarcoma.
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Affiliation(s)
- Omar Al Laham
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic..
| | - Rahaf Sharaf Aldeen
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Surgery, Al-Mouwasat University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic..
| | - Zein Ibrahim Basha
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Pathology, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic..
| | - Amad Ali
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Surgery, Al-Mouwasat University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic..
| | - Alaa Alhanwt
- Faculty of Medicine, Damascus University, Damascus, (The) Syrian Arab Republic.; Department of Surgery, Al Assad University Hospital, Damascus University, Damascus, (The) Syrian Arab Republic..
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12
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Heishima K, Aketa N, Heishima M, Kawachi A. Hemangiosarcoma in dogs as a potential non-rodent animal model for drug discovery research of angiosarcoma in humans. Front Oncol 2023; 13:1250766. [PMID: 38130992 PMCID: PMC10733437 DOI: 10.3389/fonc.2023.1250766] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2023] [Accepted: 11/06/2023] [Indexed: 12/23/2023] Open
Abstract
Since the domestication of dogs 10,000 years ago, they have shared their living environment with humans and have co-evolved. The breeding process that dogs have undergone in only a few centuries has led to a significant accumulation of specific genetic alterations that could induce particular diseases in certain breeds. These canine diseases are similar to what is found in humans with several differences; therefore, comparing such diseases occurring in humans and dogs can help discover novel disease mechanisms, pathways, and causal genetic factors. Human angiosarcoma (AS) and canine hemangiosarcoma (HSA), which are sarcomas originating from endothelium, are examples of diseases shared between humans and dogs. They exhibit similar characteristics and clinical behaviors, although with some critical differences resulting from evolution. In this review, we will describe the similarities and differences in terms of clinical and molecular characteristics between human AS and canine HSA, and discuss how these similarities and differences can be applied to advance the treatment of these diseases.
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Affiliation(s)
- Kazuki Heishima
- Institute for Advanced Study (GUiAS), Gifu University, Gifu, Japan
- Center for One Medicine Innovative Translational Research (COMIT), Gifu University, Gifu, Japan
| | - Naohiko Aketa
- Clinical and Translational Research Center, Keio University Hospital, Tokyo, Japan
| | | | - Asuka Kawachi
- Division of Cancer RNA Research, National Cancer Center, Tokyo, Japan
- Department of Medical Oncology, National Cancer Center Hospital, Tokyo, Japan
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13
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Kim WJ, Kim HK. Current understanding of angiosarcoma: disease biology and evolving treatment. Arch Craniofac Surg 2023; 24:203-210. [PMID: 37919906 PMCID: PMC10622948 DOI: 10.7181/acfs.2023.00409] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2023] [Revised: 08/28/2023] [Accepted: 09/12/2023] [Indexed: 11/04/2023] Open
Abstract
Angiosarcoma is a very rare soft tissue sarcoma that originates from endothelial cells and typically has a poor prognosis. It is most commonly found in elderly white men and can occur anywhere in the body, particularly in the head, neck, and scalp. Patients who have undergone previous radiation treatment or who have chronic lymphedema also face an elevated risk of this condition. Various genetic changes are suspected to contribute to the development of angiosarcoma, and these changes have been identified as potential targets for treatment. For localized disease, wide surgical resection is often the prudent course of action. A multidisciplinary approach, which may include surgery, radiotherapy, systemic chemotherapy, or immunotherapy, is typically the most effective way to achieve favorable outcomes. In this review, we discuss the general understanding of angiosarcoma and its management, with a particular focus on the current evolving treatments for the disease.
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Affiliation(s)
- Woo Ju Kim
- Department of Plastic and Reconstructive Surgery, Chung-Ang University Gwangmyeong Hospital, Chung-Ang University School of Medicine, Gwangmyeong, Korea
| | - Han Koo Kim
- Department of Plastic and Reconstructive Surgery, Chung-Ang University Hospital, Chung-Ang University School of Medicine, Seoul, Korea
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14
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Rhoul C, Kharkhach A, Aabdi H, Atmani A, Mhand M, Seghrouchni N, Amal B, Bouhout T, Serji B, El Harroudi T. Radiation-induced angiosarcoma of the breast: a case report. Ann Med Surg (Lond) 2023; 85:5047-5050. [PMID: 37811031 PMCID: PMC10553051 DOI: 10.1097/ms9.0000000000001141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/25/2022] [Accepted: 07/31/2023] [Indexed: 10/10/2023] Open
Abstract
Introduction Breast angiosarcoma is a rare, aggressive tumour affecting adult women. It can occur in two forms, primary form and secondary forms or radiation-induced breast angiosarcoma affecting patients with history of breast or chest radiotherapy. Case presentation The authors report a new case of breast angiosarcoma in 52-year-old women, with history of invasive ductal carcinoma, and reporting a discoloration of her breast skin. The patient did undergo a mastectomy of right breast and adjuvant chemotherapy. Conclusion/discussion Surgery with total excision associated or not to adjuvant chemotherapy remains the treatment of choice in breast angiosarcoma.
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Affiliation(s)
- Chafik Rhoul
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Ayoub Kharkhach
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Houssam Aabdi
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Abderrahman Atmani
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Mohammed Mhand
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Noura Seghrouchni
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Pathology, Mohammed VI University Hospital Oujda Morocco
| | - Bennani Amal
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Pathology, Mohammed VI University Hospital Oujda Morocco
| | - Tarik Bouhout
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Badr Serji
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
| | - Tijani El Harroudi
- Faculty of Medicine and Pharmacy, Mohammed Ist university
- Department of Oncological Surgery, Regional oncology Center
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15
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Saleh JS, Whittington CP, Bresler SC, Patel RM. Cutaneous vascular tumors: an updated review. Hum Pathol 2023; 140:53-65. [PMID: 37059271 DOI: 10.1016/j.humpath.2023.04.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2023] [Revised: 03/30/2023] [Accepted: 04/06/2023] [Indexed: 04/16/2023]
Abstract
Cutaneous vascular tumors constitute a heterogeneous group of entities that share overlapping morphologic and immunohistochemical features, which can be diagnostically challenging for pathologists and dermatopathologists. Our understanding and knowledge of vascular neoplasms have improved over time, resulting in both a refinement of their classification by the International Society for the Study of Vascular Anomalies (ISSVA) and an improvement in the accurate diagnosis and clinical management of vascular neoplasms. This review article aims to summarize the updated clinical, histopathological, and immunohistochemical characteristics of cutaneous vascular tumors, as well as to highlight their associated genetic mutations. Such entities include infantile hemangioma, congenital hemangioma, tufted angioma, spindle cell hemangioma, epithelioid hemangioma, pyogenic granuloma, Kaposiform hemangioendothelioma, retiform hemangioendothelioma, pseudomyogenic hemangioendothelioma, Kaposi sarcoma, angiosarcoma, and epithelioid hemangioendothelioma.
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Affiliation(s)
- Jasmine S Saleh
- Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA
| | | | - Scott C Bresler
- Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA; Department of Dermatology, University of Michigan, Ann Arbor, MI 48109, USA
| | - Rajiv M Patel
- Department of Pathology, University of Michigan, Ann Arbor, MI 48109, USA; Department of Dermatology, University of Michigan, Ann Arbor, MI 48109, USA; Cutaneous Pathology, WCP Laboratories, Inc., Maryland Heights, MO 63043, USA.
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16
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Benton A, Terwilliger E, Moriarty NM, Liu B, Murphy A, Maluvac H, Shu M, Gartenhaus LE, Janson ND, Pfeffer CM, Utturkar SM, Parkinson EI, Lanman NA, Hanna JA. Target gene regulatory network of miR-497 in angiosarcoma. BIORXIV : THE PREPRINT SERVER FOR BIOLOGY 2023:2023.09.24.559218. [PMID: 37808715 PMCID: PMC10557590 DOI: 10.1101/2023.09.24.559218] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/10/2023]
Abstract
Angiosarcoma (AS) is a vascular sarcoma that is highly aggressive and metastatic. Due to its rarity, treatment options for patients are limited, therefore more research is needed to identify possible therapeutic vulnerabilities. We previously found that conditional deletion of Dicer1 drives AS development in mice. Given the role of DICER1 in canonical microRNA (miRNA) biogenesis, this suggests that miRNA loss is important in AS development. After testing miRNAs previously suggested to have a tumor-suppressive role in AS, microRNA-497-5p (miR-497) suppressed cell viability most significantly. We also found that miR-497 overexpression led to significantly reduced cell migration and tumor formation. To understand the mechanism of miR-497 in tumor suppression, we identified clinically relevant target genes using a combination of RNA-sequencing data in an AS cell line, expression data from AS patients, and target prediction algorithms. We validated miR-497 direct regulation of CCND2, CDK6, and VAT1. One of these genes, VAT1, is an understudied protein that has been suggested to promote cell migration and metastasis in other cancers. Indeed, we find that pharmacologic inhibition of VAT1 with the natural product Neocarzilin A reduces AS migration. This work provides insight into the mechanisms of miR-497 and its target genes in AS pathogenesis.
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Affiliation(s)
- Annaleigh Benton
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Emma Terwilliger
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Noah M. Moriarty
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
- Department of Medicinal Chemistry and Molecular Pharmacology, Purdue University, West Lafayette, IN USA
| | - Bozhi Liu
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Ant Murphy
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Hannah Maluvac
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Mae Shu
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Lauren E. Gartenhaus
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Nimod D. Janson
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Claire M. Pfeffer
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Sagar M. Utturkar
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
| | - Elizabeth I. Parkinson
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
- Department of Medicinal Chemistry and Molecular Pharmacology, Purdue University, West Lafayette, IN USA
- Department of Chemistry, Purdue University, West Lafayette, IN, USA
| | - Nadia A. Lanman
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
- Department of Comparative Pathobiology, Purdue University, West Lafayette, IN USA
| | - Jason A. Hanna
- Department of Biological Sciences, Purdue University, West Lafayette, IN USA
- Purdue University Institute for Cancer Research, Purdue University, West Lafayette, IN USA
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17
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Dhabhar JB, Mehta V. Recurrent metastatic angiosarcoma presenting as Kasabach-Merritt syndrome. BMJ Case Rep 2023; 16:e255134. [PMID: 37500188 PMCID: PMC10387657 DOI: 10.1136/bcr-2023-255134] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/29/2023] Open
Abstract
Angiosarcoma is an incredibly rare type of malignancy, accounting for only 1%-2% of all soft-tissue sarcomas globally. It is clinically, pathologically and radiologically difficult to diagnose angiosarcoma owing to its varied presentation with little or no well-defined imaging findings.Kasabach-Merritt syndrome is also a lesser-heard entity which carries extremely poor prognosis. It is primarily seen in infants with vascular malformations and in kaposiform haemangioendothelioma. It is a condition of consumptive coagulopathy and only few of the cases have been reported so far in the adults with a background of angiosarcoma.This report presents the case of a male in his 70s who was diagnosed with metastatic angiosarcoma and experienced a complicated disease course due to Kasabach-Merritt syndrome.
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Affiliation(s)
- Jeyhan Boman Dhabhar
- Medical Oncology, PD Hinduja National Hospital and Medical Research Centre, Mumbai, India
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18
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Vaqar M, Sharif A, Iqbal N, Irfan M. Bilateral pneumothorax in a patient with angiosarcoma of the scalp: a case report. J Med Case Rep 2023; 17:173. [PMID: 37122029 PMCID: PMC10150512 DOI: 10.1186/s13256-023-03878-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/24/2022] [Accepted: 03/07/2023] [Indexed: 05/02/2023] Open
Abstract
BACKGROUND Angiosarcoma is a rare, vascular malignancy that arises from endothelial cells of blood vessels. This case report aims to create the awareness of its existence in the region and its mode of presentation. CASE PRESENTATION A 63-year-old Pakistani man presented to the emergency department with sudden bilateral chest pain and shortness of breath for 2 days. On examination, a scalp lesion was seen which had been increasing in size over the last 6 weeks. The lesion was 8 × 10 cm in size with an irregular border, non-tender, violet and dome-shaped in elevation on the right occipito-parietal lobe of the skull. Chest computed tomography (CT) showed multiple cystic lesions on both lungs, patchy areas of ground-glass opacities, nodules of variable sizes and bilateral pneumothorax. Bilateral tube thoracostomy was performed which provided symptomatic relief for shortness of breath. His bronchoalveolar lavage (BAL) was negative for infection. He underwent biopsy of scalp lesion which was positive for aggressive angiosarcoma. CONCLUSION Bilateral spontaneous pneumothorax can be the initial manifestation of aggressive cutaneous angiosarcoma and frequently leads to respiratory failure. Early recognition is essential to prevent delay in diagnosis and management.
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Affiliation(s)
- Maham Vaqar
- Department of Medicine, Aga Khan University, Karachi, Pakistan
| | - Ayesha Sharif
- Department of Medicine, Aga Khan University, Karachi, Pakistan
| | - Nousheen Iqbal
- Department of Medicine, Aga Khan University, Karachi, Pakistan
| | - Muhammad Irfan
- Department of Medicine, Aga Khan University, Karachi, Pakistan.
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19
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Conti M, Morciano F, Rossati C, Gori E, Belli P, Fornasa F, Romanucci G, Rella R. Angiosarcoma of the Breast: Overview of Current Data and Multimodal Imaging Findings. J Imaging 2023; 9:jimaging9050094. [PMID: 37233313 DOI: 10.3390/jimaging9050094] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2023] [Revised: 04/25/2023] [Accepted: 04/26/2023] [Indexed: 05/27/2023] Open
Abstract
Angiosarcoma of the breast is a rare breast cancer, which can arise de novo (primary breast angiosarcoma, PBA) or as a secondary malignancy (secondary breast angiosarcoma, SBA) as a result of a biological insult. In the latter case, it is usually diagnosed in patients with a previous history of radiation therapy following a conserving treatment for breast cancer. Over the years, the advances in early diagnosis and treatment of breast cancer, with increasing use of breast-conserving surgery and radiation therapy (instead of radical mastectomy), brought about an increased incidence of the secondary type. PBA and SBA have different clinical presentations and often represent a diagnostic challenge due to the nonspecific imaging findings. The purpose of this paper is to review and describe the radiological features of breast angiosarcoma, both in conventional and advanced imaging to guide radiologists in the diagnosis and management of this rare tumor.
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Affiliation(s)
- Marco Conti
- UOC di Radiologia Toracica e Cardiovascolare, Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo A. Gemelli 8, 00168 Rome, Italy
| | - Francesca Morciano
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Claudia Rossati
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Elisabetta Gori
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Paolo Belli
- UOC di Radiologia Toracica e Cardiovascolare, Dipartimento di Diagnostica per Immagini, Radioterapia Oncologica ed Ematologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Largo A. Gemelli 8, 00168 Rome, Italy
- Facoltà di Medicina e Chirurgia, Università Cattolica Sacro Cuore, Largo F. Vito 1, 00168 Rome, Italy
| | - Francesca Fornasa
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Giovanna Romanucci
- UOSD Breast Unit ULSS9, Ospedale di Marzana, Piazzale Lambranzi, 1, 37142 Verona, Italy
| | - Rossella Rella
- UOC Diagnostica per Immagini, Ospedale G.B. Grassi, Via Gian Carlo Passeroni, 28, 00122 Rome, Italy
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20
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El-Ghazzi N, Bay JO. [Rare tumors: Angiosarcoma]. Bull Cancer 2023; 110:581-588. [PMID: 36973135 DOI: 10.1016/j.bulcan.2023.03.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2022] [Revised: 02/21/2023] [Accepted: 03/02/2023] [Indexed: 03/29/2023]
Abstract
Angiosarcomas are a rare subtype representing 1-2% of soft tissue sarcomas. Risk factors are rarely elucidated but radiotherapy and lymphedema are the most common ones, usually following local treatment for local breast cancer. Despite the improvement of our knowledge, the prognosis remains poor with 35-40% of 5 year-overall survival. Local treatment when feasible should include a R0 surgery completed with adjuvant radiation. When metastatic, front lines chemotherapies include doxorubicine or weekly paclitaxel. If possible, in oligometastatic patients, metastasectomy should always be considered allowing the best responses. The knowledge of angiosarcoma's biology is rapidly increasing and new biomarkers are emerging. The use of immunotherapy in particular subtypes including head and neck angiosarcomas shows promising results. The model of the angiosarcoma project, a patient-participating study, seems to be an excellent way to study rare tumors. We should focus our efforts on understanding the underlying molecular biology to propose the best precision medicine for those patients.
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Affiliation(s)
- Nathan El-Ghazzi
- CHU Gabriel-Montpied, service d'oncologie médicale, Clermont-Ferrand, France; Université Clermont Auvergne, Clermont-Ferrand, France.
| | - Jacques-Olivier Bay
- CHU Gabriel-Montpied, service d'oncologie médicale, Clermont-Ferrand, France; Université Clermont Auvergne, Clermont-Ferrand, France
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21
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Kamitani R, Matsumoto K, Takeda T, Mizuno R, Oya M. Evaluation of prognostic factors and treatment options for renal angiosarcoma: A retrospective analysis of 113 reported cases. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2023; 49:263-270. [PMID: 36116986 DOI: 10.1016/j.ejso.2022.09.001] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/20/2022] [Revised: 08/29/2022] [Accepted: 09/02/2022] [Indexed: 01/24/2023]
Abstract
BACKGROUND Primary renal angiosarcoma (PRA) is an extremely rare and aggressive neoplasm. Indeed, due to its rarity, established clinical guidelines for PRA have not yet been established. In this study, we attempted to investigate its prognostic factors and treatment options. METHODS We systematically searched for articles describing PRA that had been published up until December 2021. The final cohort included 113 patients in 103 articles. The starting point of this study was the time of diagnosis and the end point was the time of recurrence and disease-specific mortality. RESULTS Metastasis at diagnosis was associated with poorer disease-specific survival (DSS) (p = 0.001). Tumors of more than 5 cm had poorer DSS than tumors of 5 cm or less (p < 0.001). Multivariate analysis demonstrated that primary metastatic status, and tumor size were independent prognostic factors. In cases of localized PRA, tumor sizes exceeding 5 cm had also prognostic significance for recurrence-free survival (RFS) and DSS. Surgical margins, postoperative radiation, and postoperative systemic therapy were not associated with prognoses. However, in a subgroup analysis of tumors exceeding 5 cm, postoperative radiation therapy improved RFS and DSS (p = 0.022 and p = 0.031, respectively). In cases of metastatic PRA, systemic therapy improved DSS (p < 0.001). CONCLUSION We identified several prognostic factors for PRA. Among them, primary metastatic status and tumor size exceeding 5 cm were selected as independent prognostic factors. Postoperative radiation therapy for large, localized PRA and systemic therapy for recurrent and metastatic PRA might be a treatment option.
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Affiliation(s)
- Rei Kamitani
- Keio University School of Medicine, Department of Urology, Japan
| | | | - Toshikazu Takeda
- Keio University School of Medicine, Department of Urology, Japan
| | - Ryuichi Mizuno
- Keio University School of Medicine, Department of Urology, Japan
| | - Mototsugu Oya
- Keio University School of Medicine, Department of Urology, Japan
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22
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Russell DH, Montgomery EA, Susnik B. Low to Intermediate (Borderline) Grade Breast Spindle Cell Lesions on Needle Biopsy: Diagnostic Approach and Clinical Management. Adv Anat Pathol 2022; 29:309-323. [PMID: 35838633 DOI: 10.1097/pap.0000000000000353] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
Spindle cell proliferations of the breast are a heterogeneous group of lesions ranging from benign or reactive lesions to aggressive malignant neoplasms. Diagnosis on core biopsy can be particularly challenging as lesions displaying different lineages associated with variable outcomes share overlapping morphologies (scar vs. fibromatosis-like metaplastic carcinoma) whereas individual entities can exhibit a large variety of appearances (myofibroblastoma). In this review, lesions are grouped into lineage, when possible, including those showing fibroblastic/myofibroblastic differentiation, ranging from entities that require no additional management, such as scar and nodular fasciitis, to those with unpredictable clinical outcomes such as fibromatosis and solitary fibrous tumor or locally aggressive behavior such as dermatofibrosarcoma protuberans. The review of low-grade vascular lesions includes atypical vascular lesion and low-grade angiosarcoma. Also discussed are various adipocytic lesions ranging from lipoma to liposarcoma, and rare smooth muscle and neural entities more commonly encountered in locations outside the breast, such as leiomyoma, neurofibroma, schwannoma, or granular cell tumor. Optimal histological evaluation of these entities merges clinical and radiologic data with morphology and ancillary testing. We present our approach to immunohistochemical and other ancillary testing and highlight issues in pathology correlation with imaging. Recent updates in the management of breast spindle cell lesions are addressed. In a well-sampled lesion with radiographic concordance, the core biopsy diagnosis reliably guides management and we advocate the inclusion of management recommendations in the pathology report. Precise characterization using up to date guidelines is important to identify a subset of patients who may safely avoid unnecessary surgical procedures. A multidisciplinary approach with close collaboration with our clinical colleagues is emphasized.
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Affiliation(s)
- Daniel H Russell
- Departments of Pathology University of Miami Hospital and Jackson Health Systems, Miami, FL
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23
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Conforti F, Gronchi A, Penel N, Jones RL, Broto JM, Sala I, Bagnardi V, Napolitano A, Pala L, Pennacchioli E, Catania C, Queirolo P, Grigani G, Merlini A, Stacchiotti S, Comandone A, Vincenzi B, Quagliuolo V, Bertuzzi A, Boglione A, Palassini E, Baldi GG, Blay JY, Ryckewaert T, Toulmonde M, Italiano A, Le Cesne A, Ray-Coquard I, Cruz J, Hernández-León CN, Trufero JM, da Silva Moura D, Muñiz NH, De Pas T. Chemotherapy in patients with localized angiosarcoma of any site: A retrospective european study. Eur J Cancer 2022; 171:183-192. [PMID: 35728378 DOI: 10.1016/j.ejca.2022.04.030] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2022] [Revised: 04/21/2022] [Accepted: 04/28/2022] [Indexed: 11/24/2022]
Abstract
BACKGROUND We retrospectively investigated the role of (neo)adjuvant chemotherapy in patients with primary, localized angiosarcoma. METHODS We selected all patients with primary, localized angiosarcoma, who had received radical surgery between January 2005 and December 2019 at 33 European sarcoma reference centers. The primary objective was to compare the outcome of patients who received (neo)adjuvant chemotherapy versus those who did not, in terms of overall survival (OS), disease-free survival (DFS) and distant metastasis-free survival (DMFS). To reduce the risk of confounding due to imbalance, a propensity-score matching(PSM) was performed. Finally, subgroups analysis was performed according to tumor site, tumor size (< 50 mm or ≥ 50 mm) and patients predicted 10-years OS according to the nomogram sarculator (two different cutoff-values were applied: ≤ 33% or > 33% and < 60% or ≥ 60%). RESULTS 362 patients were analyzed: 149 (41.2%; treated group) received (neo) adjuvant chemotherapy and 213 (58.6%; control group) did not. The median follow-up for the OS endpoint was 5.1 years (95% CI: 4.0-5.5). The OS-HR was 0.58 (95%CI: 0.40-0.83; p-value = 0.003) in the univariate analysis and 0.74 (95% CI: 0.38-1.43; p = 0.367) in the PSM analysis. The DFS-HR was 0.75 (95% CI: 0.57-0.98; p-value = 0.036) in the univariate analysis, and 0.91 (95% CI:0.56-1.48; p-value = 0.7) in the PSM analysis. The DMFS-HR was 0.75 (95% CI: 0.55-1.02; p-value = 0.065) in univariate analysis and 0.92 (95% CI: 0.53-1.61; p-value = 0.769) in the PSM analysis. Subgroup analysis revealed no heterogeneity of results in strata of tumor site. On the contrary, there was a trend for heterogeneity according to tumor size and patient's risk of death. For all the endpoints analyzed, patients with tumors smaller than 50 mm or at lower risk of death seem to have no benefit from chemotherapy, while patients with larger tumors or at higher risk of death at 10 years seem to derive substantial benefit. CONCLUSION This large, retrospective study suggests that patients affected by > 50 mm and/or high-risk primary, localized angiosarcoma could benefit from (neo)adjuvant chemotherapy.
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Affiliation(s)
- Fabio Conforti
- Division of Melanoma, Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy.
| | | | - Nicholas Penel
- Lille University and Centre Oscar Lambret, Lille, France
| | - Robin L Jones
- Sarcoma Unit, Royal Marsden NHS Foundation Trust and Institute of Cancer Research, London, United Kingdom
| | - Javier M Broto
- Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD), Madrid, Spain; Medical Oncology Department, Hospital Fundación Jimenez Diaz University Hospital, Madrid, Spain; General de Villalba University Hospital, Madrid, 28400, Spain; Autonomous University of Madrid, Madrid, Spain
| | - Isabella Sala
- Department of Statistics and Quantitative Methods, University of Milan-Bicocca, Milan, Italy
| | - Vincenzo Bagnardi
- Department of Statistics and Quantitative Methods, University of Milan-Bicocca, Milan, Italy
| | - Andrea Napolitano
- Sarcoma Unit, Royal Marsden NHS Foundation Trust and Institute of Cancer Research, London, United Kingdom
| | - Laura Pala
- Division of Melanoma, Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy
| | - Elisabetta Pennacchioli
- Division of Melanoma, Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy
| | - Chiara Catania
- Division of Thoracic Oncology, European Institute of Oncology, Milan, Italy
| | - Paola Queirolo
- Division of Melanoma, Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy
| | - Giovanni Grigani
- Candiolo Cancer Institute, FPO-IRCCS, 10060 Candiolo, Turin, Italy
| | - Alessandra Merlini
- Candiolo Cancer Institute, FPO-IRCCS, 10060 Candiolo, Turin, Italy; Department of Oncology, University of Turin, Turin, Italy
| | | | | | - Bruno Vincenzi
- Medical Oncology Department, University Campus Bio-Medico, 00128 Rome, Italy
| | | | - Alexia Bertuzzi
- Medical Oncology and Hematology Unit, Humanitas Clinical and Research Center, IRCCS, Rozzano, Italy
| | | | - Elena Palassini
- Fondazione IRCCS Istituto Nazionale Dei Tumori, Milan, Italy
| | - Giacomo G Baldi
- "Sandro Pitigliani" Medical Oncology Department, Hospital of Prato, Prato, Italy
| | - Jean-Yves Blay
- Centre Léon Bérard & University Cl. Bernard Lyon I, EURACAN, LYRICAN, Lyon, France
| | | | - Maud Toulmonde
- Department of Medicine, Institut Bergonié, Bordeaux, Nouvelle-Aquitaine
| | - Antoine Italiano
- Department of Medicine, Institut Bergonié, Bordeaux, Nouvelle-Aquitaine
| | - Axel Le Cesne
- Department of Medical Oncology, Gustave Roussy, Villejuif, France
| | - Isabelle Ray-Coquard
- Centre Leon Bérard, Hesper Lab, EA 7425, Université Claude Bernard Lyon Est, Lyon, France
| | - Josefina Cruz
- Oncology Department, University Hospital of Canary Islands, Canary Islands, Spain
| | | | - Javier M Trufero
- Oncology Department, University Hospital Miguel Servet, Zaragoza, Spain
| | - David da Silva Moura
- Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD), Madrid, Spain; Autonomous University of Madrid, Madrid, Spain
| | - Nadia H Muñiz
- Instituto de Investigacion Sanitaria Fundacion Jimenez Diaz (IIS/FJD), Madrid, Spain; Medical Oncology Department, Hospital Fundación Jimenez Diaz University Hospital, Madrid, Spain; General de Villalba University Hospital, Madrid, 28400, Spain; Autonomous University of Madrid, Madrid, Spain
| | - Tommaso De Pas
- Division of Melanoma, Sarcomas and Rare Tumors, European Institute of Oncology, Milan, Italy
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24
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Arts L, Roesti A, Haller C, Danzer D. Angiosarcoma after popliteal aneurysm exclusion surgery. A systematic review. Ann Vasc Surg 2022; 86:482-489. [PMID: 35820530 DOI: 10.1016/j.avsg.2022.06.012] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/08/2022] [Revised: 05/30/2022] [Accepted: 06/02/2022] [Indexed: 11/27/2022]
Abstract
OBJECTIVE Expansion after popliteal artery aneurysm exclusion with bypass is a common phenomenon. Popliteal angiosarcoma is seldom reported in literature and in most cases after popliteal artery aneurysm surgery. This paper aims to present the case of a popliteal angiosarcoma, initially diagnosed as late aneurysmal growth after exclusion surgery, to conduct a systematic review of popliteal angiosarcoma and assess any association between angiosarcoma and previous popliteal aneurysm surgery. METHODS We performed a secondary popliteal aneurysmorraphy through posterior approach for symptomatic aneurysm expansion in a 79-year-old woman, 9 years after medial femoropopliteal venous bypass and aneurysm exclusion. The postoperative course was complicated by recurrent hematomas and wound spillages requiring multiple revisions. Pathological analysis identified an angiosarcoma. Staging revealed bone invasion and pulmonary metastasis. Despite transfemoral amputation and adjuvant chemotherapy the patient died eight months later. We performed a systematic review through MEDLINE on 'primary' and 'secondary' (with previous vascular surgery) angiosarcoma in popliteal artery aneurysm. Research was done using the terms '(hem)angiosarcoma', 'aneurysm', 'popliteal aneurysm or artery', 'femoral aneurysm or artery'. Other soft tissue sarcoma or non-popliteal locations were excluded. RESULTS Including this case, only 13 angiosarcomas in popliteal aneurysms are currently described. Two were reported without previous surgery considered a primary angiosarcoma and 11 after popliteal artery aneurysm surgery (secondary angiosarcoma). Patient age ranges from 8 months to 83 years with a male predominance (10/3). Nine of the 11 patients with secondary angiosarcoma were initially diagnosed as popliteal aneurysm expansion after previous bypass surgery, the two other secondary cases presented respectively with pain and inflammatory syndrome without expansion. All prior surgical exclusion were carried out by a medial approach. Interval with the index operation ranges from 3 months to 15 years. Death was reported in 8 of the 13 cases within the first year of diagnosis. CONCLUSION Although seldom reported, popliteal angiosarcomas are mainly described after popliteal artery aneurysm exclusion surgery, raising suspicion on a potential association, yet causality cannot be demonstrated. Angiosarcoma should be included in the differential diagnosis of popliteal aneurysm growth or unexpected outcome after exclusion bypass surgery. Systematic imaging and pathological studies should be undertaken to allow early diagnosis and treatment. Routine use of a posterior approach, with aneurysm resection, when feasible as initial popliteal artery aneurysm treatment, might reduce the risk of late sarcomatous transformation.
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Affiliation(s)
- Laure Arts
- Department of vascular surgery, Centre Hospitalier du Valais Romand, Switzerland.
| | - Aurore Roesti
- Department of vascular surgery, Centre Hospitalier du Valais Romand, Switzerland
| | - Claude Haller
- Department of vascular surgery, Centre Hospitalier du Valais Romand, Switzerland
| | - Daniel Danzer
- Department of vascular surgery, Centre Hospitalier du Valais Romand, Switzerland
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25
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Radiation-induced angiosarcoma of the vagina and vulva: Case report and review of literature. Gynecol Oncol Rep 2022; 41:100990. [PMID: 35540025 PMCID: PMC9079236 DOI: 10.1016/j.gore.2022.100990] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2022] [Revised: 04/21/2022] [Accepted: 04/26/2022] [Indexed: 11/24/2022] Open
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26
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Messina V, Cope B, Keung EZ, Fiore M. Management of Skin Sarcomas. Surg Oncol Clin N Am 2022; 31:511-525. [DOI: 10.1016/j.soc.2022.03.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/18/2022]
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27
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Goerdt LV, Schneider SW, Booken N. Kutane Angiosarkome: molekulare Pathogenese und neue therapeutische Ansätze. J Dtsch Dermatol Ges 2022; 20:429-444. [PMID: 35446507 DOI: 10.1111/ddg.14694_g] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2021] [Accepted: 11/16/2021] [Indexed: 12/27/2022]
Abstract
Das kutane Angiosarkom (CAS) ist ein hochaggressiver maligner Tumor mit schlechter Prognose. Das primäre, spontane CAS (pCAS) und das sekundäre, mit einer Bestrahlung oder einem Lymphödem assoziierte CAS (sCAS) unterscheiden sich klinisch sowie molekular. Die Amplifikation/Überexpression von Myc ist ein charakteristisches, wenn auch nicht ausschließliches Merkmal von sCAS, während der Verlust von TP53 selektiv bei pCAS vorkommt. Detaillierte molekulare Analysen mit modernen Multi-Omics-Ansätzen haben gezeigt, dass sowohl pCAS als auch sCAS eine erhebliche molekulare Heterogenität aufweisen. Die betroffenen Gene und ihre molekularen Regulatoren sind mögliche therapeutische Zielstrukturen. Darüber hinaus kann das pCAS in Cluster mit hoher Mutationsrate und/oder ausgeprägten Entzündungssignaturen eingeteilt werden, die als Grundlage für die künftige Stratifizierung von pCAS-Patienten in immuntherapeutischen klinischen Studien dienen können. Während die Aufklärung der der Erkrankung zugrunde liegenden molekularen Veränderungen zügig voranschreitet, verläuft die Entwicklung daraus abgeleiteter neuer Therapien für das CAS jedoch bisher eher langsam. Dennoch wurden einige über die Standardtherapien wie Operation und Radiochemotherapie hinausgehende klinische Studien zu neuen Behandlungsmöglichkeiten initiiert. Dazu gehören zielgerichtete Therapien gegen VEGF und VEGFR1-3 wie Bevacizumab und Pazopanib, sowie β-Adrenozeptorenblocker wie Propranolol. Derzeit werden auch Immuntherapien entwickelt, unter anderem unter Verwendung der Immuncheckpoint-Inhibitoren Pembrolizumab und Nivolumab sowie des Anti-RANKL-Antikörper Denosumab.
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Affiliation(s)
- Lea V Goerdt
- Klinik für Dermatologie und Venerologie, Universitätsklinikum Hamburg-Eppendorf (UKE), Hamburg.,Asklepios Campus Hamburg, medizinische Fakultät, Semmelweis Universität Budapest, Hamburg
| | - Stefan W Schneider
- Klinik für Dermatologie und Venerologie, Universitätsklinikum Hamburg-Eppendorf (UKE), Hamburg
| | - Nina Booken
- Klinik für Dermatologie und Venerologie, Universitätsklinikum Hamburg-Eppendorf (UKE), Hamburg
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28
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Kashihara T, Igaki H, Ogata D, Nakayama H, Nakamura S, Okuma K, Mori T, Yamakawa K, Takahashi A, Namikawa K, Takahashi A, Takahashi K, Kaneda T, Inaba K, Murakami N, Nakayama Y, Okamoto H, Yamazaki N, Itami J. Prognostic factor analysis of definitive radiotherapy using intensity-modulated radiation therapy and volumetric modulated arc therapy with boluses for scalp angiosarcomas. Sci Rep 2022; 12:4355. [PMID: 35288619 PMCID: PMC8921322 DOI: 10.1038/s41598-022-08362-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/26/2021] [Accepted: 03/07/2022] [Indexed: 11/24/2022] Open
Abstract
Cutaneous angiosarcomas is a rare cancer with poor prognoses. The common radiotherapy techniques that have been reported so far are two pairs of lateral X-ray and electron fields. However, it is quite difficult to irradiate scalp angiosarcomas (SAs) homogeneously with this technique. In this study, safety, effectiveness, and risk factors were assessed for localized SAs ≥ 5 cm treated with intensity-modulated radiotherapy (IMRT) or volumetric modulated arc therapy (VMAT) with boluses. Sixty-eight angiosarcoma patients who had received radiotherapy in our institution between January 2007 and November 2020 were retrieved from our radiotherapy database. Of these patients, 27 localized SA patients were included in the retrospective analysis. The 2-year overall survival, local progression-free rate, and distant metastases-free survival were 41.8%, 48.4%, and 33.1%. All the patients experienced acute radiation dermatitis ≥ grade 2, with18 (66.7%) ≥ grade 3. No nodule lesion was a significant unfavorable predictive factor of acute radiation dermatitis ≥ grade 3. Tumor bleeding at the initiation of radiotherapy and tumor invasion to the face were significant predictive factors of overall survival, and tumor bleeding at the initiation of radiotherapy was also a significant predictive factor of local progression-free rate.
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29
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Veterano C, Teixeira S, Silva I, Coelho A, Almeida R, Pinto PS. Angiosarcoma of a Femoropopliteal Bypass Thrombosed Vein Graft. Vasc Endovascular Surg 2022; 56:448-453. [PMID: 35258356 DOI: 10.1177/15385744221077824] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
BACKGROUND Angiosarcoma is a rare subtype of malignant vascular tumours which has been only anecdotally described in patients submitted to lower limb revascularization. CASE PRESENTATION This paper reports a patient previously submitted to a femoropopliteal bypass using autologous great saphenous vein (GSV). Nine years after the initial surgery, a primary angiosarcoma of the thrombosed vein graft was diagnosed, requiring en bloc surgical resection. Rampant metastatic spread was documented despite primary tumour surgical resection with a dismal outcome within months. CONCLUSIONS Malignant transformation of autologous vein for lower limb revascularization is extremely rare and anecdotally described in the literature. It is a poorly studied complication with an aggressive behaviour. This report further reinforces the need for early recognition of this pathology.
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Affiliation(s)
- Carlos Veterano
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal
| | - Sérgio Teixeira
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal.,Kidney Transplant Program, Transplantation Department, Centro Hospitalar Universitário do Porto, Porto, Portugal
| | - Ivone Silva
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal.,Kidney Transplant Program, Transplantation Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.,89239Instituto de Ciências Biomédicas Abel Salazar - Universidade do Porto, Porto, Portugal
| | - Andreia Coelho
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal
| | - Rui Almeida
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal.,Kidney Transplant Program, Transplantation Department, Centro Hospitalar Universitário do Porto, Porto, Portugal.,89239Instituto de Ciências Biomédicas Abel Salazar - Universidade do Porto, Porto, Portugal
| | - Pedro Sá Pinto
- Angiology and Vascular Surgery Department, 112085Centro Hospitalar Universitário do Porto, Porto, Portugal.,Kidney Transplant Program, Transplantation Department, Centro Hospitalar Universitário do Porto, Porto, Portugal
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30
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Hendrick LE, Zambetti BR, Wong DL, Dickson PV, Glazer ES, Shibata D, Fleming MD, Tsao M, Portnoy DC, Deneve JL. Visceral angiosarcoma: A nationwide analysis of treatment factors and outcomes. J Surg Oncol 2022; 125:1231-1237. [PMID: 35234280 DOI: 10.1002/jso.26826] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2021] [Accepted: 02/03/2022] [Indexed: 11/06/2022]
Abstract
BACKGROUND AND OBJECTIVES Visceral angiosarcoma is rare and aggressive, accounting for 2% of soft tissue sarcomas. Using a national data set, we examine determinants of outcomes for patients presenting with this rare disease. METHODS The 2004-2015 National Cancer Database was queried for patients with visceral angiosarcoma. Trends in treatment and outcomes were examined. Factors affecting overall survival (OS) were assessed with log-rank and Cox regression. RESULTS Eight hundred and ninety-three patients with visceral angiosarcoma were identified (median age 65 years, male [63%], Charlson comorbidity index <1 [86%]). Tumor size was <5 cm in 20.7%, and 34.2% were moderate/high grade. Median OS was 3.8 months (95% CI: 3.4-4.4). By multivariate analysis, increased tumor grade and size, and liver/biliary origin demonstrated worse OS while surgery, radiation, and systemic chemotherapy demonstrated improved OS (all p < 0.001). Survival was similar between patients achieving R0 resection and those with R1/2 resection receiving chemotherapy by Kaplan-Meier analysis. CONCLUSIONS Visceral angiosarcomas are rare tumors with poor outcomes. Liver/biliary origin, higher tumor grade, and larger tumor size demonstrate worse outcomes. While R0 resection remains the mainstay of treatment, patients with R1/R2 resection have improved survival with addition of chemotherapy. Consideration should be made for multimodal therapy in these patients.
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Affiliation(s)
- Leah E Hendrick
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Benjamin R Zambetti
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Denise L Wong
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Paxton V Dickson
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Evan S Glazer
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - David Shibata
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Martin D Fleming
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - Miriam Tsao
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
| | - David C Portnoy
- Department of Medical Oncology, West Cancer Center and Research Institute, Memphis, Tennessee, USA
| | - Jeremiah L Deneve
- Department of Surgery, Division of Surgical Oncology, University of Tennessee Health Science Center, Memphis, Tennessee, USA
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Sakamoto S, Okabayashi T, Tabuchi M, Sui K, Murokawa T, Iwata J. Epithelioid angiosarcoma of the duodenum: a case report. Surg Case Rep 2022; 8:35. [PMID: 35224706 PMCID: PMC8882486 DOI: 10.1186/s40792-022-01391-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2022] [Accepted: 02/22/2022] [Indexed: 12/04/2022] Open
Abstract
Background Angiosarcomas are rare malignant tumors that arise from the endothelium of blood vessels. They occur most commonly in the skin and soft tissue, and less commonly in the breast, liver, bone, and spleen. Gastrointestinal angiosarcomas are extremely rare. Herein, we present a case of duodenal epithelioid angiosarcoma that was treated with surgical resection. Case presentation A 68-year-old man presented with a 1-month history of fatigue and hypotension. He visited the outpatient clinic for a routine follow-up. Laboratory examination revealed anemia. Esophagogastroduodenoscopy revealed multiple duodenal lesions with central ulceration. A biopsy showed a sheet-like arrangement of large round and spindle-shaped tumor cells that were positive for CD31. Based on the histological and immunohistochemical staining findings, an epithelioid angiosarcoma was diagnosed. Computed tomography (CT) and positron emission tomography–CT revealed no lymph node metastasis or distant metastasis. Radical subtotal stomach-preserving pancreatoduodenectomy with lymphadenectomy was performed. After removing the specimen, reconstruction was performed using the Child procedure. Grossly, two dark-red polypoid tumors were found in the second portion of the duodenum. Histological evaluation revealed proliferation of malignant round and polygonal cells arranged in sheets and spindle-like cells arranged in bundles. Vasoformative structures were recognized as slit-like spaces containing red blood cells. Immunohistochemical staining demonstrated that the tumor cells were positive for CD31. These findings confirmed the diagnosis of epithelioid angiosarcoma in the duodenum. The patient’s postoperative course was uneventful. The patient was discharged on postoperative day 19 without any complications. At a follow-up examination in the outpatient clinic at postoperative 4 months, no evidence of recurrence was detected. Conclusion The present report describes a case of duodenal epithelioid angiosarcoma. Duodenal angiosarcomas may cause anemia and gastrointestinal bleeding. Because angiosarcomas sometimes show epithelioid cytomorphology, immunohistochemical analysis is useful for confirming the diagnosis.
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Affiliation(s)
- Shinya Sakamoto
- Department of Gastroenterological Surgery at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan
| | - Takehiro Okabayashi
- Department of Gastroenterological Surgery at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan.
| | - Motoyasu Tabuchi
- Department of Gastroenterological Surgery at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan
| | - Kenta Sui
- Department of Gastroenterological Surgery at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan
| | - Takahiro Murokawa
- Department of Gastroenterological Surgery at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan
| | - Jun Iwata
- Department of Diagnostic Pathology at Kochi Health Sciences Center, 2125-1 Ike, Kochi-City, Kochi, 781-8555, Japan
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32
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Goerdt LV, Schneider SW, Booken N. Cutaneous Angiosarcomas: Molecular Pathogenesis Guides Novel Therapeutic Approaches. J Dtsch Dermatol Ges 2022; 20:429-443. [PMID: 35218306 DOI: 10.1111/ddg.14694] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/10/2021] [Accepted: 11/16/2021] [Indexed: 12/12/2022]
Abstract
Cutaneous angiosarcoma (CAS) is a highly aggressive cancer with a poor prognosis. Primary, spontaneous CAS (pCAS) and secondary, post-irradiation- or lymphedema-associated CAS (sCAS) are clinically, but also molecularly distinct. Myc amplification/overexpression is a characteristic, although not exclusive feature of sCAS, while loss of TP53 selectively occurs in pCAS. Detailed molecular analyses with modern multi-omics approaches have revealed that both pCAS and sCAS exhibit considerable molecular heterogeneity. Affected genes and their molecular regulators including a plethora of microRNAs may serve as future drug targets. Furthermore, pCAS could be subdivided into clusters with high tumor mutational burden and/or high tumor inflammation signatures providing a rationale for the stratification of pCAS patients in future immunotherapeutic clinical studies. Development of novel treatment regimens guided by these molecular alterations, however, cannot fully keep up with the pace of their discovery due to the low incidence of the disease. Nevertheless, beyond conventional surgery and chemoradiotherapy, clinical trials investigating novel treatment options have been initiated including targeted therapies against VEGF and VEGFR1-3 such as bevacizumab and pazopanib, and β-adrenoreceptor blockers such as propranolol. Finally, immunotherapies are being developed including immune checkpoint inhibitors pembrolizumab and nivolumab as well as anti-RANKL antibody denosumab.
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Affiliation(s)
- Lea V Goerdt
- Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.,Asklepios Campus Hamburg, Medical Faculty, Semmelweis University Budapest, Hamburg, Germany
| | - Stefan W Schneider
- Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany
| | - Nina Booken
- Department of Dermatology and Venereology, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany
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Bhaludin BN, Thway K, Adejolu M, Renn A, Kelly-Morland C, Fisher C, Jones RL, Messiou C, Moskovic E. Imaging features of primary sites and metastatic patterns of angiosarcoma. Insights Imaging 2021; 12:189. [PMID: 34921641 PMCID: PMC8684573 DOI: 10.1186/s13244-021-01129-9] [Citation(s) in RCA: 14] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/24/2021] [Accepted: 11/14/2021] [Indexed: 12/14/2022] Open
Abstract
Angiosarcomas are rare, aggressive soft tissue sarcomas originating from endothelial cells of lymphatic or vascular origin and associated with a poor prognosis. The clinical and imaging features of angiosarcomas are heterogeneous with a wide spectrum of findings involving any site of the body, but these most commonly present as cutaneous disease in the head and neck of elderly men. MRI and CT are complementary imaging techniques in assessing the extent of disease, focality and involvement of adjacent anatomical structures at the primary site of disease. CT plays an important role in the evaluation of metastatic disease. Given the wide range of imaging findings, correlation with clinical findings, specific risk factors and patterns of metastatic disease can help narrow the differential diagnosis. The final diagnosis should be confirmed with histopathology and immunohistochemistry in combination with clinical and imaging findings in a multidisciplinary setting with specialist sarcoma expertise. The purpose of this review is to describe the clinical and imaging features of primary sites and metastatic patterns of angiosarcomas utilising CT and MRI.
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Malignant Vascular Tumors of the Head and Neck-Which Type of Therapy Works Best? Cancers (Basel) 2021; 13:cancers13246201. [PMID: 34944821 PMCID: PMC8699575 DOI: 10.3390/cancers13246201] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2021] [Revised: 12/03/2021] [Accepted: 12/07/2021] [Indexed: 12/13/2022] Open
Abstract
Simple Summary Malignant vascular tumors are extremely rare tumors with variable clinical courses, and few data on their clinical management are available. Diagnosis is difficult due to their wide morphologic appearance. The intent of the present review is to demonstrate the current knowledge and management on malignant vascular tumors of the head and neck area. The mainstay of treatment for malignant vascular tumors is surgery, but radiotherapy and chemotherapy are also parts of the treatment concept especially in angiosarcomas. Targeted therapy, antiangiogenetic drugs and immunotherapy have been studied as new treatment options. Abstract Malignant vascular tumors of the head and neck are rare neoplasms with variable clinical presentation, wide age distribution, and variable clinical courses. The heterogeneous presentation of angiosarcomas and epithelioid hemangioendothelioma often leads to misdiagnosis and unsuitable treatment. While risk factors for angiosarcomas are previous radiation, chronic lymphedema, and exposure to arsenic, thorium oxide, or vinyl chloride, there are only limited and retrospective data available on prognostic factors in EHE. In both angiosarcomas and EHE, surgery is the mainstay of treatment. There is limited evidence regarding the role of radiotherapy in EHE, although EHE is considered relatively radiosensitive. In angiosarcomas, adjuvant radiotherapy is recommended according to retrospective case series. A standard medical therapy for metastasized malignant vascular tumors is lacking. Chemotherapy, which is effective in angiosarcoma, is mostly ineffective in EHE. Targeted therapy, antiangiogenetic drugs and immunotherapy have been studied as new treatment options. The goal of this review is to summarize the current data regarding malignant vascular tumors along with their diagnosis and management.
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Chaudhry IUH. Radical surgical resection of giant Angiosarcoma of the posterior Mediastinum: A rare neoplasm with rare presentation as epigastric pain. Ann Med Surg (Lond) 2021; 72:103087. [PMID: 34868576 PMCID: PMC8626570 DOI: 10.1016/j.amsu.2021.103087] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2021] [Revised: 11/16/2021] [Accepted: 11/16/2021] [Indexed: 11/27/2022] Open
Abstract
Angiosarcoma of the Posterior Mediastinum is a rare entity. We herein report a case of a giant posterior mediastinal Angiosarcoma. A 54-year female presented with a one-year history of epigastric pain. Her upper gastrointestinal tract endoscopy revealed no abnormality. Chest x-Ray showed a shadow in the hilar area. A computed tomographic scan of the Thorax (CT) and MRI Scan showed a mass in the Posterior Mediastinum. CT-guided biopsy revealed the tumor of vascular origin. The tumor was completely resected. Post-operative recovery was uneventful. After 14 months follow up patient is disease-free, and a CT scan of the chest showed no recurrence.
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Liu YY, Xu BS, Pan QZ, Weng DS, Zhang X, Peng RQ. New nomograms to predict overall and cancer-specific survival of angiosarcoma. Cancer Med 2021; 11:74-85. [PMID: 34786885 PMCID: PMC8704180 DOI: 10.1002/cam4.4425] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/17/2021] [Revised: 10/12/2021] [Accepted: 10/14/2021] [Indexed: 12/25/2022] Open
Abstract
Objective This study was designed to establish and validate promising and reliable nomograms for predicting the survival of angiosarcoma (AS) patients. Methods The Surveillance, Epidemiology, and End Results database was queried to collect the clinical information of 785 AS patients between 2004 and 2015. Data were split into a training cohort (n = 549) and a validation cohort (n = 236) without any preference. Univariate Cox and multivariate Cox regression analyses were performed to analyze the clinical parameters. Independent prognostic factors were then identified. Two nomograms were constructed to predict overall survival (OS) and cancer‐specific survival (CSS) at 3 and 5 years. Finally, the models were evaluated using concordance indices (C‐indices), calibration plots, and decision curve analysis (DCA). Results Based on the inclusion and exclusion criteria, 785 individuals were included in this analysis. Univariate and multivariate Cox regression analyses revealed that age, tumor size, and stage were prognostic factors independently associated with the OS of AS. Tumor site, tumor size, and stage were associated with the CSS of AS. Based on the statistical results and clinical significance of variables, nomograms were built. The nomograms for OS and CSS had C‐indices of 0.666 and 0.654, respectively. The calibration curves showed good agreement between the predictive values and the actual values. DCA also indicated that the nomograms were clinically useful. Conclusion We established nomograms with good predictive ability that could provide clinicians with better predictions about the clinical outcomes of AS patients.
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Affiliation(s)
- Yuan-Yuan Liu
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
| | - Bu-Shu Xu
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
| | - Qiu-Zhong Pan
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
| | - De-Sheng Weng
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
| | - Xing Zhang
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
| | - Rui-Qing Peng
- Melanoma and Sarcoma Medical Oncology Unit, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-Sen University Cancer Center, Guangzhou, China.,State Key Laboratory of Oncology in South China, Sun Yat-Sen University Cancer Center, Guangzhou, China
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Negură I, Bădescu MC, Rezuş C, Dănilă R, Florescu AF, Blaj M, Moroşan E, Apostol DGC. Morphology and one immunohistochemical marker are enough for diagnosis of primary thyroid angiosarcoma. Arch Clin Cases 2021; 8:7-13. [PMID: 34754933 PMCID: PMC8565704 DOI: 10.22551/2021.30.0801.10178] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Primary thyroid angiosarcoma is a very rare and extremely aggressive mesenchymal malignant neoplasm showing morphological and immunophenotypic evidence of endothelial cell differentiation. Early diagnosis of this tumor along with radical thyroidectomy followed by postoperative radiotherapy and chemotherapy are essential for adequate management of the patient. Currently available data on diagnosis and treatment options of this neoplasm are limited because it is a rare disease in endocrine organs. Raising awareness regarding its diagnosis can help to optimize the treatment and to improve the survival of the patient. We present the case of a 72-year-old female patient with multiple comorbidities who addressed to the hospital with obstructive respiratory symptoms: dyspnea, wheezing and hoarseness. The investigations, both clinical and paraclinical, identified a local invasive cervical mass located mainly in the left thyroid lobe, whose immunohistochemical examination confirmed primary thyroid angiosarcoma. Although this type of neoplasm is described mainly in the Alpine regions, it can appear in lower altitude regions and this tumor needs to be differentiated from a high-grade neoplasm (anaplastic thyroid carcinoma).
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Affiliation(s)
- Ion Negură
- Pathology Department, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania
| | - Minerva Codruţa Bădescu
- Department of Internal Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania.,III Internal Medicine Clinic, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania
| | - Ciprian Rezuş
- Department of Internal Medicine, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania.,III Internal Medicine Clinic, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania
| | - Radu Dănilă
- 3 Surgical Department, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania
| | | | - Mihaela Blaj
- Department of Anesthesia and Intensive Care, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania
| | - Eugenia Moroşan
- Pathology Department, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania
| | - Delia Gabriela Ciobanu Apostol
- Pathology Department, "Sf. Spiridon" Emergency County Clinical Hospital, Iasi, Romania.,Pathology Department, "Grigore T. Popa" University of Medicine and Pharmacy, Iasi, Romania
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Sharon CE, Straker RJ, Shannon AB, Shabason JE, Zhang PJL, Fraker DL, Miura JT, Karakousis GC. Neoadjuvant radiation for cutaneous and soft tissue angiosarcoma. J Surg Oncol 2021; 125:509-515. [PMID: 34689338 DOI: 10.1002/jso.26727] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2021] [Accepted: 10/16/2021] [Indexed: 11/05/2022]
Abstract
BACKGROUND AND OBJECTIVES Neoadjuvant radiation (NRT) is frequently utilized in soft tissue sarcomas to increase local control. Its utility in cutaneous and soft tissue angiosarcoma remains poorly defined. METHODS This retrospective cohort study was performed using the National Cancer Database (2004-2016) evaluating patients with clinically localized, surgically resected angiosarcomas. Factors associated with receipt of NRT in the overall cohort and margin positivity in treatment naïve patients were identified by univariate and multivariable logistic regression analyses. Survival was assessed using Kaplan-Meier analysis. RESULTS Of 597 patients, 27 (4.5%) received NRT. Increasing age (odds ratio [OR] 0.95, p = 0.025), tumor size more than or equal to 5 cm (OR 3.16, p = 0.02), and extremity tumor location (OR 3.99, p = 0.04) were associated with receipt of NRT. All patients who received NRT achieved an R0 resection (p = 0.03) compared with 17.9% of patients without NRT. Factors associated with risk of margin positivity included tumor size more than or equal to 5 cm (OR 1.85, p = 0.01), and head/neck location (OR 2.24, p = 0.006). NRT was not significantly associated with improved survival (p = 0.21). CONCLUSIONS NRT improves rates of R0 resection but is infrequently utilized in cutaneous and soft tissue angiosarcoma. Increased usage of NRT, particularly for patients with lesions more than or equal to 5 cm, or head and neck location, may help achieve complete resections.
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Affiliation(s)
- Cimarron E Sharon
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Richard J Straker
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Adrienne B Shannon
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Jacob E Shabason
- Department of Radiation Oncology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Paul J L Zhang
- Department of Pathology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Douglas L Fraker
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - John T Miura
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
| | - Giorgos C Karakousis
- Department of Surgery, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA
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Mao J, Hu J, Chen Y, Li Y, Run X. Development and validation of a prognostic nomogram model in primary cutaneous and subcutaneous soft tissue angiosarcoma. J DERMATOL TREAT 2021; 33:2466-2474. [PMID: 34429003 DOI: 10.1080/09546634.2021.1968333] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
OBJECTIVE The current study aimed to investigate the prognosis and treatment of primary cutaneous angiosarcoma (PCA) and primary subcutaneous angiosarcoma (PSCA), and tried to develop a prognostic nomogram model of them. METHODS A total of 1763 cases retrieved from the Surveillance, Epidemiology, and End Results (SEER) database were retrospectively analyzed. Survival analyses were performed to explore the prognosis of patients and the effects of different treatment methods. All data were randomly allocated into a training set and a testing set to develop and validate the nomogram model. RESULTS The findings showed that age, sex, grade, tumor size, multiple primary malignant tumors, stage, primary site surgery (PSS), radiotherapy (RT), and chemotherapy (CT) were correlated with prognosis (p < .05). The nomogram achieved good accuracy in predicting the prognosis. PSS + RT + CT showed the best prognosis for patients in stages I, II, and III (p < .05). CONCLUSION PCA and PSCA are rare with poor prognoses. Patients undergoing PSS may not gain survival benefits from combining with RT or (and) CT, whereas PSS + RT + CT should be actively performed in earlier stages to improve the prognosis of patients. The nomogram model can be used to predict the overall survival rate and guide better treatment.
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Affiliation(s)
- Jinqian Mao
- Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, WuHan, China
| | - Jin Hu
- Department of Breast and Thyroid Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, WuHan, China
| | - Yunfei Chen
- Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, WuHan, China
| | - Yiqing Li
- Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, WuHan, China
| | - Xiaoqin Run
- Department of Vascular Surgery, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, WuHan, China
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Constantinidou A, Sauve N, Stacchiotti S, Blay JY, Vincenzi B, Grignani G, Rutkowski P, Guida M, Hindi N, Klein A, Thibaud V, Sufliarsky J, Desar I, Steeghs N, Litiere S, Gelderblom H, Jones RL. Evaluation of the use and efficacy of (neo)adjuvant chemotherapy in angiosarcoma: a multicentre study. ESMO Open 2021; 5:S2059-7029(20)32651-X. [PMID: 32847839 PMCID: PMC7451279 DOI: 10.1136/esmoopen-2020-000787] [Citation(s) in RCA: 31] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/13/2020] [Revised: 06/04/2020] [Accepted: 06/06/2020] [Indexed: 01/30/2023] Open
Abstract
Introduction Angiosarcomas constitute approximately 2% to 3% of all soft tissue sarcomas, are characterised by an aggressive clinical behaviour and poor outcome. Optimal management of localised angiosarcomas consists of complete surgical resection with or without radiation. However, due to the infiltrating nature of this disease, complete resection is often not possible. Despite optimal management, the outcome of patients with localised disease remains poor. The role of (neo)adjuvant chemotherapy in angiosarcomas remains undefined. The aim of this study is to document the outcome of patients treated with (neo)adjuvant chemotherapy and assess the feasibility of performing a prospective trial by evaluating the number of patients treated at sarcoma referral centres. Methods A retrospective search within participating EORTC (European Organisation for Research and Treatment of Cancer) sites for patients treated with (neo)adjuvant chemotherapy was made. Patients treated between January 2007 and January 2016 were included. Results A total of 15 institutions participated and 86 patients were evaluable, 43 were treated with neoadjuvant, 27 with adjuvant chemotherapy and 16 with both. At the time of analysis, the median follow-up from diagnosis was 4.6 years. Median overall survival (OS) was 4.9 years (2.9 N) and the percentage alive at 4 years was 57.9 (45.5 to 68.4). The median disease-free survival was 1.4 years (0.9 to 1.7) and the percentage disease-free at 4 years was 26.8% (17.9 to 36.5). Conclusion The outcome of angiosarcoma patients treated with (neo)adjuvant chemotherapy in this case series compares favourably with previously published data. Due to the aggressive nature of angiosarcoma, a prospective trial of neoadjuvant chemotherapy should be considered.
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Affiliation(s)
- Anastasia Constantinidou
- University of Cyprus Medical School, Nicosia, Cyprus.,Medical Oncology, Bank of Cyprus Oncology Center, Strovolos, Cyprus
| | - Nicolas Sauve
- Department of Biostatistics, EORTC, Brussels, Belgium
| | - Silvia Stacchiotti
- Medical Oncology, IRCCS Fondazione Istituto Nazionale Tumori, Milan, Italy, Milan, Italy
| | - Jean-Yves Blay
- Medical Oncology, Centre Léon Bérard, Lyon, Rhône-Alpes, France.,Université Claude Bernard Lyon 1, Villeurbanne, France
| | - Bruno Vincenzi
- Medical Oncology, Universita Campus Bio-Medico di Roma, Roma, Lazio, Italy
| | - Giovanni Grignani
- Division of Medical Oncology, Candiolo Cancer Institute - FPO, IRCCS, Torino, Italy
| | - Piotr Rutkowski
- Soft Tissue/Bone Sarcoma and Melanoma, Maria Sklodowska-Curie National Research Institute of Oncology, Warsaw, Poland
| | - Michele Guida
- Medical Oncology, IRCCS Istituto Tumori "Giovanni Paolo II", Bari, Italy
| | - Nadia Hindi
- Instituto de Biomedicina de Sevilla, IBiS/Hospital Universitario Virgen del Rocío/CSIC/Universidad de Sevilla, Sevilla, Spain.,Medical Oncology, Hospital Universitario Virgen del Rocio, Sevilla, Spain
| | - Alexander Klein
- Department of Orthopaedics, Physical Medicine and Rehabilitation University Hospital, LMU, Munich, Germany
| | - Valentin Thibaud
- Medical Oncology, Institut Claudius Regaud, Institut Universitaire du Cancer de Toulouse, Toulouse, France, Toulouse, France
| | - Jozef Sufliarsky
- Oncology, National Cancer Institute, Bratislava, Slovakia, Bratislava, Slovakia
| | - Ingrid Desar
- Medical Oncology, Radboud University Medical Centre Nijmegen, Nijmegen, Netherlands, Nijmegen, Gelderland, The Netherlands
| | - Neeltje Steeghs
- Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
| | | | - Hans Gelderblom
- Medical Oncology, Leiden University Medical Center, Leiden, The Netherlands
| | - Robin L Jones
- Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, London, UK .,Insitute of Cancer Research, London, UK
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Schöffski P, Timmermans I, Wildiers H, Dumez H, Hompes D, Christiaens M, Sciot R, Laenen A, Lee CJ, Meyskens T. Retrospective Analysis of the Clinical Presentation, Treatment and Outcome of Angiosarcoma in a Sarcoma Referral Center. Oncol Res Treat 2021; 44:322-332. [PMID: 33946082 DOI: 10.1159/000516000] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/04/2021] [Accepted: 03/18/2021] [Indexed: 11/19/2022]
Abstract
INTRODUCTION Angiosarcoma (AS) is a rare subtype of soft tissue sarcoma. We performed a retrospective analysis of patient characteristics, treatments and prognostic factors in patients treated in a single sarcoma center. METHODS We reviewed records of patients treated between 1987 and 2018, categorized in 7 different subtypes according to tissue of origin and underlying risk factors. The Kaplan-Meier method was used to estimate overall survival (OS); the Cox proportional hazards model was used to study prognostic variables. RESULTS Among 134 patients, 30% had radiation-induced, 31% primary soft tissue, 24% cutaneous, 5% breast, 4% bone, 2% lymphedema-associated and 4% unknown primary AS. Key patient/disease characteristics varied between subgroups. The median OS was 22.0 months for the entire cohort, with 28.9% with a 5-year survival. Metastasis at diagnosis was seen in 23% of patients; 38% developed metachronous metastasis. Sixty-six (49%) patients received systemic therapy; common first-line treatments were doxorubicin (48%) and paclitaxel (39%), without a significant difference in OS between agents. Younger age, breast/radiation-induced AS, primary surgery and palliative chemotherapy were associated with better OS. Synchronous metastasis, soft tissue/unknown primary location correlated with poor survival. CONCLUSION AS is a very heterogeneous sarcoma subtype, with substantial variability in clinical presentation and survival among patient subsets. Prognosis is poor, and there is no difference in outcome comparing the 2 most frequently used chemotherapy agents in the first line, paclitaxel and doxorubicin.
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Affiliation(s)
- Patrick Schöffski
- Department of General Medical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Iris Timmermans
- Department of General Medical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Hans Wildiers
- Department of General Medical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Herlinde Dumez
- Department of General Medical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Daphne Hompes
- Department of Surgical Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Melissa Christiaens
- Department of Radiotherapy/Oncology, University Hospitals Leuven, Leuven, Belgium
| | - Raf Sciot
- Department of Pathology, University Hospitals Leuven, Leuven, Belgium
| | - Annouschka Laenen
- Leuven Center of Biostatistics and Statistic Bioinformatics, KU Leuven, Leuven, Belgium
| | - Che-Jui Lee
- Laboratory of Experimental Oncology, Department of Oncology, KU Leuven, Leuven, Belgium
| | - Thomas Meyskens
- Department of General Medical Oncology, University Hospitals Leuven, Leuven, Belgium
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Jeyaraman M, Muthu S, Prabhakar M, Jeyaraman N, Agarwal G, Jain R. Primary Angiosarcoma of Humerus - A Case Report and Literature Review. J Orthop Case Rep 2021; 11:12-17. [PMID: 34557431 PMCID: PMC8422013 DOI: 10.13107/jocr.2021.v11.i05.2186] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/08/2023] Open
Abstract
INTRODUCTION Osseous angiosarcoma is a very rare tumor of bone with aggressive behavior, propensity for recurrences, and distant metastasis. The etiology of osseous angiosarcoma is uncertain; however, specific risk factors have been recognized. The diagnosis of angiosarcoma of bone demands multimodality imaging in conjunction with histopathological and vascular marker evaluation to aptly differentiate them from other vascular tumors. Treatment of osseous angiosarcoma remains controversial. CASE REPORT A 53-year-old male presented with pain and swelling of the right upper 1/3rd of the arm following heaviness while lifting weight for 3 months. He had a history of significant weight loss and appetite with no history of inciting trauma or irradiation in the past. On examination, a diffuse swelling was noted in the right shoulder and right scapular aspect with varied consistency and ill-defined borders and margins. The skin over the swelling was stretched and shiny with dilated engorged veins over it. The plain radiograph of the right shoulder with humerus revealed a large expansile lytic soft-tissue mass in the right proximal humerus with a wide zone of transition without sclerotic margins. Magnetic resonance imaging showed T1 hypointense, T2/PDFS hyperintense large well-defined expansile lytic lesion with multi-loculated cysts, and multiple blood-fluid levels involving right proximal humerus. The patient underwent an incisional biopsy which exhibited angiosarcoma of the humerus. The patient was treated with six cycles of chemotherapy with a mesna, doxorubicin, ifosfamide, and dacarbazine regimen. The patient was still under follow-up. CONCLUSION Being a rare clinical entity, controversy exists in angiosarcoma of humerus regarding its etiology and recommended management protocols. Histopathology and immunohistochemistry remain the gold standard in differentiating osseous angiosarcoma from other osseous vascular tumors. The treatment protocol has to be standardized to decrease morbidity and to improve the functional quality of life of the patient.
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Affiliation(s)
- Madhan Jeyaraman
- Department of Orthopedics, School of Medical Sciences and Research, Sharda University, Greater Noida, Uttar Pradesh. India
| | - Sathish Muthu
- Department of Orthopaedics, Government Medical College and Hospital, Dindigul, Tamil Nadu. India
- Address of Correspondence: Dr. Sathish Muthu, Department of Orthopaedics, Government Medical College and Hospital, Dindigul, Tamil Nadu. India. E-mail:
| | - Manoj Prabhakar
- Department of Radio-Diagnosis, Osmania Medical College, Hyderabad, Telangana. India
| | - Naveen Jeyaraman
- Department of Orthopedics, Kasturba Medical College, MAHE University, Manipal, Karnataka. India
| | - Garima Agarwal
- Department of Pathology, School of Medical Sciences and Research, Sharda University, Greater Noida, Uttar Pradesh. India
| | - Rashmi Jain
- Department of Orthopedics, School of Medical Sciences and Research, Sharda University, Greater Noida, Uttar Pradesh. India
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Pediatric Metastatic Cardiac Angiosarcoma Successfully Treated With Multimodal Therapy: Case Report and Review of Literature. J Pediatr Hematol Oncol 2021; 43:e203-e206. [PMID: 31725539 DOI: 10.1097/mph.0000000000001674] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2019] [Accepted: 10/24/2019] [Indexed: 11/27/2022]
Abstract
Cardiac angiosarcoma (AS) is an extremely rare, malignant vascular tumor with <10 cases reported in the pediatric literature. Prognosis is dismal with overall survival often <1 year from initial diagnosis. In this report, we present the case of a 10-year-old boy with metastatic cardiac AS who is currently alive and is the longest pediatric survivor of metastatic cardiac AS reported in the literature. This is the only published pediatric case to successfully use a combination of surgical resection, conventional chemotherapy, radiation and targeted therapies including bevacizumab and pazopanib for metastatic cardiac AS.
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Mizuno T, Tomita N, Takaoka T, Tomida M, Fukuma H, Tsuchiya T, Shibamoto Y. Dosimetric Comparison of Helical Tomotherapy, Volumetric-Modulated Arc Therapy, and Intensity-Modulated Proton Therapy for Angiosarcoma of the Scalp. Technol Cancer Res Treat 2021; 20:1533033820985866. [PMID: 33517860 PMCID: PMC7871283 DOI: 10.1177/1533033820985866] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/18/2022] Open
Abstract
Objective: We compared radiotherapy plans among helical tomotherapy (HT),
volumetric-modulated arc therapy (VMAT), and intensity-modulated proton
therapy (IMPT) for angiosarcoma of the scalp (AS). Methods: We conducted a planning study for 19 patients with AS. The clinical target
volume (CTV) 1 and CTV2 were defined as the gross tumor volume with a
specific margin and total scalp, respectively. For HT and VMAT, the planning
target volume (PTV) 1 and PTV2 were defined as CTV1 and CTV2 with 0.5-cm
margins, respectively. For IMPT, robust optimization was used instead of a
CTV-PTV margin (i.e. CTV robust). The targets of the HT and VMAT plans were
the PTV, whereas the IMPT plans targeted the CTV robust. In total, 70 Gy and
56 Gy were prescribed as the D95% (i.e. dose to 95% volume) of PTV1 (or CTV1
robust) and PTV2 (or CTV2 robust), respectively, using the simultaneous
integrated boost (SIB) technique. Other constraint goals were also defined
for the target and organs at risk (OAR). Results: All dose constraint parameters for the target and OAR met the goals within
the acceptable ranges for the 3 techniques. The coverage of the targets
replaced by D95% and D98% were almost equivalent among the 3 techniques. The
homogeneity index of PTV1 or CTV1 robust was equivalent among the 3
techniques, whereas that of PTV2 or CTV2 robust was significantly higher in
the IMPT plans than in the other plans. IMPT reduced the Dmean of the brain
and hippocampus by 49% to 95%, and the Dmax of the spinal cord, brainstem,
and optic pathway by 70% to 92% compared with the other techniques. Conclusion: The 3 techniques with SIB methods provided sufficient coverage and
satisfactory homogeneity for the targets, but IMPT achieved the best OAR
sparing.
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Affiliation(s)
- Tomoki Mizuno
- Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
| | - Natsuo Tomita
- Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
| | - Taiki Takaoka
- Narita Memorial Proton Center, Toyohashi, Aichi, Japan
| | | | - Hiroshi Fukuma
- Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
| | - Takahiro Tsuchiya
- Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
| | - Yuta Shibamoto
- Department of Radiology, Nagoya City University Graduate School of Medical Sciences, Nagoya, Aichi, Japan
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Saha J, Kim JH, Amaya CN, Witcher C, Khammanivong A, Korpela DM, Brown DR, Taylor J, Bryan BA, Dickerson EB. Propranolol Sensitizes Vascular Sarcoma Cells to Doxorubicin by Altering Lysosomal Drug Sequestration and Drug Efflux. Front Oncol 2021; 10:614288. [PMID: 33598432 PMCID: PMC7882688 DOI: 10.3389/fonc.2020.614288] [Citation(s) in RCA: 24] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/05/2020] [Accepted: 12/14/2020] [Indexed: 01/02/2023] Open
Abstract
Angiosarcoma is a rare cancer of blood vessel-forming cells with a high patient mortality and few treatment options. Although chemotherapy often produces initial clinical responses, outcomes remain poor, largely due to the development of drug resistance. We previously identified a subset of doxorubicin-resistant cells in human angiosarcoma and canine hemangiosarcoma cell lines that exhibit high lysosomal accumulation of doxorubicin. Hydrophobic, weak base chemotherapeutics, like doxorubicin, are known to sequester within lysosomes, promoting resistance by limiting drug accessibility to cellular targets. Drug synergy between the beta adrenergic receptor (β-AR) antagonist, propranolol, and multiple chemotherapeutics has been documented in vitro, and clinical data have corroborated the increased therapeutic potential of propranolol with chemotherapy in angiosarcoma patients. Because propranolol is also a weak base and accumulates in lysosomes, we sought to determine whether propranolol enhanced doxorubicin cytotoxicity via antagonism of β-ARs or by preventing the lysosomal accumulation of doxorubicin. β-AR-like immunoreactivities were confirmed in primary tumor tissues and cell lines; receptor function was verified by monitoring downstream signaling pathways of β-ARs in response to receptor agonists and antagonists. Mechanistically, propranolol increased cytoplasmic doxorubicin concentrations in sarcoma cells by decreasing the lysosomal accumulation and cellular efflux of this chemotherapeutic agent. Equivalent concentrations of the receptor-active S-(-) and -inactive R-(+) enantiomers of propranolol produced similar effects, supporting a β-AR-independent mechanism. Long-term exposure of hemangiosarcoma cells to propranolol expanded both lysosomal size and number, yet cells remained sensitive to doxorubicin in the presence of propranolol. In contrast, removal of propranolol increased cellular resistance to doxorubicin, underscoring lysosomal doxorubicin sequestration as a key mechanism of resistance. Our results support the repurposing of the R-(+) enantiomer of propranolol with weak base chemotherapeutics to increase cytotoxicity and reduce the development of drug-resistant cell populations without the cardiovascular and other side effects associated with antagonism of β-ARs.
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Affiliation(s)
- Jhuma Saha
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States
| | - Jong Hyuk Kim
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States.,Animal Cancer Care and Research Program, College of Veterinary Medicine University of Minnesota, St. Paul, MN, United States.,Masonic Cancer Center, University of Minnesota, Minneapolis, MN, United States
| | - Clarissa N Amaya
- Department of Biomedical Sciences, Texas Tech University Health Sciences Center, El Paso, TX, United States.,Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, United States
| | - Caleb Witcher
- Department of Biology, Stephen F. Austin State University, Nacogdoches, TX, United States
| | - Ali Khammanivong
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States
| | - Derek M Korpela
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States
| | - David R Brown
- Department of Veterinary and Biomedical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States
| | - Josephine Taylor
- Department of Biology, Stephen F. Austin State University, Nacogdoches, TX, United States
| | - Brad A Bryan
- Department of Biomedical Sciences, Texas Tech University Health Sciences Center, El Paso, TX, United States.,Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, United States
| | - Erin B Dickerson
- Department of Veterinary Clinical Sciences, College of Veterinary Medicine, University of Minnesota, St. Paul, MN, United States.,Animal Cancer Care and Research Program, College of Veterinary Medicine University of Minnesota, St. Paul, MN, United States.,Masonic Cancer Center, University of Minnesota, Minneapolis, MN, United States
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46
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Park C, Kim M, Kwak Y, Moon KC, Kim SH, Keam B, Kim YJ, Kim TM, Kim DW. Real-World Clinical Outcomes and Prognostic Factors for Patients with Advanced Angiosarcoma who Received Systemic Treatment. Cancer Res Treat 2021; 53:1195-1203. [PMID: 33540493 PMCID: PMC8524033 DOI: 10.4143/crt.2020.1337] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/21/2020] [Accepted: 01/30/2021] [Indexed: 12/25/2022] Open
Abstract
Purpose Angiosarcoma is a highly aggressive mesenchymal tumor. Although systemic chemotherapy is often considered for the inoperable or metastatic angiosarcoma, the outcome of such treatment is unsatisfactory and poorly delineated. Materials and Methods We reviewed electronic medical records of 75 patients with angiosarcoma who were treated with systemic chemotherapy for inoperable or metastatic disease. Patients were classified as having liver involvement if they had either primary or metastatic hepatic lesions. Results Among the patients evaluated, 51 patients were male (68%) and 24 patients (32%) had primary cutaneous angiosarcoma. Liver involvement was present in 28 patients (37.3%). A total of 59 patients received first-line weekly paclitaxel (wPac) and showed an objective response rate (ORR) of 23.7% (n=14), a median progression free survival (mPFS) of 4.0 months (95% confidence interval [CI] 3.0-6.1), and a median overall survival (mOS) of 10.2 months (95% CI 7.0-14.6). Among patients without liver involvement, patients receiving wPac (n=35) had significantly prolonged mPFS (5.8 vs. 3.2 months, respectively, p=0.014) with a tendency for prolonged mOS (13.8 vs. 11.6 months, respectively, p=0.13) than those receiving other regimens (n=12). A total of 24 patients received second- or later-line pazopanib monotherapy and showed an ORR of 16.7% (n=4), a mPFS of 2.4 months (95% CI 1.8-4.3) and a mOS of 5.4 months (95% CI 3.5-NA). Conclusion Treatment with first-line wPac and later-line pazopanib seems to provide survival benefit, especially for patients with advanced angiosarcoma without liver involvement.
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Affiliation(s)
- Changhee Park
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
| | - Miso Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.,Cancer Research Institute, Seoul National University, Seoul, Korea
| | - Yoonjin Kwak
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | - Kyung Chul Moon
- Department of Pathology, Seoul National University Hospital, Seoul, Korea
| | - Se Hyun Kim
- Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Bhumsuk Keam
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.,Cancer Research Institute, Seoul National University, Seoul, Korea
| | - Yu Jung Kim
- Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea
| | - Tae Min Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.,Cancer Research Institute, Seoul National University, Seoul, Korea
| | - Dong-Wan Kim
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea.,Cancer Research Institute, Seoul National University, Seoul, Korea
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Kehagias D, Kostopoulou E, Ravazoula P, Panagopoulos K. Thyroid angiosarcoma (TAS) - A rare diagnosis not to be missed. Clin Case Rep 2021; 9:173-176. [PMID: 33489155 PMCID: PMC7813081 DOI: 10.1002/ccr3.3492] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/30/2020] [Revised: 09/04/2020] [Accepted: 09/05/2020] [Indexed: 11/18/2022] Open
Abstract
Raising awareness about primary TAS, a rare and aggressive mesenchymal tumor, is important so that early diagnosis and undelayed radical surgery along with complementary radiation are possible. Extending the existing knowledge on the course and final outcome of the disease may help identify the best treatment approach to improve survival.
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Affiliation(s)
| | - Eirini Kostopoulou
- Division of Pediatric EndocrinologyDepartment of PediatricsUniversity Hospital of PatrasRioGreece
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Machado I, Giner F, Lavernia J, Cruz J, Traves V, Requena C, Llombart B, López-Guerrero JA, Llombart-Bosch A. Angiosarcomas: histology, immunohistochemistry and molecular insights with implications for differential diagnosis. Histol Histopathol 2021; 36:3-18. [PMID: 32885407 DOI: 10.14670/hh-18-246] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Abstract
Angiosarcomas (AS) represent a heterogenous group of tumors with variable clinical presentation. AS share an important morphologic and immunohistochemical overlap with other sarcomas, hence the differential diagnosis is challenging, especially in poorly-differentiated tumors. Although molecular studies provide significant clues, especially in the differential diagnosis with other vascular neoplasms, a thorough hematoxylin and eosin analysis remains an essential tool in AS diagnosis. In this review, we discuss pathological and molecular insights with emphasis on implications for differential diagnosis in cutaneous, breast, soft tissue and visceral AS.
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Affiliation(s)
- Isidro Machado
- Pathology Department, Instituto Valenciano de Oncología, Valencia, Spain
- Pathology Department, Hospital Quirón, Valencia, Spain.
| | - Francisco Giner
- Pathology Department, University Hospital La Fe, Valencia, Spain
| | - Javier Lavernia
- Department of Oncology, Instituto Valenciano de Oncología, Valencia, Spain
| | - Julia Cruz
- Pathology Department, Instituto Valenciano de Oncología, Valencia, Spain
| | - Víctor Traves
- Pathology Department, Instituto Valenciano de Oncología, Valencia, Spain
| | - Celia Requena
- Dermatology Department, Instituto Valenciano de Oncología, Valencia, Spain
| | - Beatriz Llombart
- Dermatology Department, Instituto Valenciano de Oncología, Valencia, Spain
| | - José Antonio López-Guerrero
- Laboratory of Molecular Biology, Instituto Valenciano de Oncología, Valencia, Spain
- IVO-CIPF Joint Research Unit of Cancer, Príncipe Felipe Research Center (CIPF), Valencia, Spain
- Department of Pathology, School of Medicine, Catholic University of Valencia 'San Vicente Mártir', Valencia, Spain
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Thyroid Angiosarcoma-Rare Case or Hard to Find. CURRENT HEALTH SCIENCES JOURNAL 2020; 46:433-437. [PMID: 33717519 PMCID: PMC7948022 DOI: 10.12865/chsj.46.04.15] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Figures] [Subscribe] [Scholar Register] [Received: 08/08/2020] [Accepted: 12/11/2020] [Indexed: 11/18/2022]
Abstract
INTRODUCTION Thyroid Angiosarcoma is a rare malignant condition of the thyroid gland with higher incidence reported in mountainous Alpine regions (Switzerland, Austria and northern Italy), featured with poor prognosis. CASE PRESENTATION A 76 years old male patient presented in our hospital complaining about difficulty in breathing, altered voice (hoarseness), fatigue and dysphagia. Careful observation of the frontal neck region revealed hemorrhaging petechiae and purpura, while palpation indicated a large nodule movable with deglutition, presumably derived by the thyroid gland. Serum biochemical thyroid function tests were normal. Ultrasound of the thyroid gland showed enlargement of the right lobe with a nodular lesion. FNAC indicated a "suspicious for malignancy" lesion. After patient's consensus, total thyroidectomy took place accompanied by excision of infiltrated infrahyoid muscles. The cytopathologic results were positive for primary thyroid angiosarcoma. Patient's postoperative condition was regular, but he died of disease progression 6 months later. DISCUSSION Angiosarcomas are malignant neoplasms arising from endothelial cells of blood vessels. They are most commonly found in skin, soft tissue, breast, bone, liver and spleen. Angiosarcomas tend to be highly hemorrhaging and invasive. Thyroid gland is a rare location of development. Cytopathologically they are grossly characterized by freely anastomosing vascular channels lined by atypical endothelial cells unusually enlarged, often multinucleated with many nucleoli and vacuoles into the cytoplasm containing fragments of erythrocytes. The immunohistological identity of angiosarcomas are endothelial line markers (CD31, CD34 and vimentin). Surgical excision when feasible is the first line treatment while adjuvant radio-and/or chemo-therapy are ambiguous. Infiltration of surrounding tissues and distant metastasis (lymph nodes and lungs) are negative prognostic factors.
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50
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Zheng CH, Wang Y, Liu TT, Ding XY, Qu JJ, Su ZD. Is Pathologic Complete Response the Surrogate in Primary Gastric Angiosarcoma Undergoing Doxorubicin-Based Neoadjuvant Chemotherapy? A Case Report. Int J Gen Med 2020; 13:1515-1521. [PMID: 33363400 PMCID: PMC7754096 DOI: 10.2147/ijgm.s280375] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2020] [Accepted: 11/24/2020] [Indexed: 11/23/2022] Open
Abstract
Introduction Angiosarcoma is a malignant tumor with low incidence. Especially in the advanced tumors, there is still a lack of knowledge of evidence-based medicine. Case Presentation We report a case of a 55-year-old woman with abdominal pain of 2 months of duration, which had increased in severity for 2 weeks prior to the presentation. The diagnosis is primary gastric angiosarcoma. We performed multiple disciplinary team (MDT), and doxorubicin-based neoadjuvant chemotherapy (NAC) was proposed. After two cycles of NAC, a computed tomography (CT) scan showed complete regression compared with the previous scan. An open surgery was done, and surgical specimens were confirmed as a pathological complete response (PCR) by pathological and immunohistochemical examination, but unfortunately, the patient suffered a relapse after the surgery in 3 months. Conclusion Repeated endoscopic biopsy and biopsy specimen examinations can improve accuracy in diagnosis. It seems that NAC could be a candidate for advanced primary gastric angiosarcomas. But after the rapid relapse, we are wondering whether pathologic complete response is the surrogate in primary gastric angiosarcoma undergoing NAC.
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Affiliation(s)
- Chun-Hui Zheng
- Cheeloo College of Medicine, Shandong University, Jinan, Shandong, People's Republic of China.,Department of Oncology Surgery, Weifang People's Hospital, Weifang, Shandong, People's Republic of China
| | - Yang Wang
- Department of General Surgery, Weifang People's Hospital, Weifang, Shandong, People's Republic of China
| | - Ting-Ting Liu
- Department of Breast Surgery, Tai'an Central Hospital, Tai'an, Shandong, People's Republic of China
| | - Xiao-Yan Ding
- Department of Laboratory Medicine, Weifang Medical University, Weifang, Shandong, People's Republic of China
| | - Jian-Jun Qu
- Department of Oncology Surgery, Weifang People's Hospital, Weifang, Shandong, People's Republic of China
| | - Zhi-De Su
- Department of Pharmacy, Weifang People's Hospital, Weifang, Shandong, People's Republic of China
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