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Yang C, Chen L, Xie X, Wu C, Wang Q. Three-dimensional (3D)-printed custom-made titanium ribs for chest wall reconstruction post-desmoid fibromatosis resection. Comput Assist Surg (Abingdon) 2025; 30:2456303. [PMID: 39840821 DOI: 10.1080/24699322.2025.2456303] [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: 01/23/2025] Open
Abstract
Desmoid fibromatosis (DF) is a rare low-grade benign myofibroblastic neoplasm that originates from fascia and muscle striae. For giant chest wall DF, surgical resection offer a radical form of treatment and the causing defects usually need repair and reconstruction, which can restore the structural integrity and rigidity of the thoracic cage. The past decade witnessed rapid advances in the application of various prosthetic material in thoracic surgery. However, three-dimensional (3D)-printed custom-made titanium ribs have never been reported for chest wall reconstruction post-DF resection. Here, we report a successful implantation of individualized 3D-printed titanium ribs to repair the chest wall defect in a patient with DF.
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Affiliation(s)
- Chen Yang
- Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China
| | - Lei Chen
- Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China
| | - Xiangyu Xie
- Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China
| | - Changping Wu
- Department of Tumor Biological Treatment, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China
| | - Qianyun Wang
- Department of Thoracic Surgery, The First People's Hospital of Changzhou, The Third Affiliated Hospital of Soochow University, Changzhou, China
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Taqi K, Stockley C, Wood M, Przybojewski S, Bouchard-Fortier A, Mack L. Cryotherapy in the Treatment of Extra-Abdominal Desmoid Tumors-A Review. Curr Oncol 2025; 32:137. [PMID: 40136341 PMCID: PMC11941758 DOI: 10.3390/curroncol32030137] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/03/2024] [Revised: 01/21/2025] [Accepted: 02/26/2025] [Indexed: 03/27/2025] Open
Abstract
The management of desmoid tumors (DTs) often poses challenges due to their variable clinical behavior, with treatment options including active surveillance, systemic therapy, and local therapies including surgery, ablation, and radiation. More recently, cryotherapy has emerged as a promising localized treatment for DTs. We aimed to conduct a review of the indications, techniques, and outcomes of cryotherapy in the treatment of extra-abdominal DTs. The review suggests that cryotherapy can be effectively used for both curative and debulking purposes, with a significant number of patients achieving symptom relief, disease stabilization, or regression. Although generally safe, cryotherapy is associated with potential risks, particularly when critical structures are in proximity. Overall, cryotherapy offers a viable, minimally invasive treatment option for DTs, with favorable outcomes in both symptom relief and tumor control.
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Affiliation(s)
- Kadhim Taqi
- Division of Surgical Oncology, Department of Oncology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada; (K.T.); (C.S.); (M.W.); (A.B.-F.)
| | - Cecily Stockley
- Division of Surgical Oncology, Department of Oncology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada; (K.T.); (C.S.); (M.W.); (A.B.-F.)
| | - Melissa Wood
- Division of Surgical Oncology, Department of Oncology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada; (K.T.); (C.S.); (M.W.); (A.B.-F.)
| | - Stefan Przybojewski
- Department of Radiology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada;
| | - Antoine Bouchard-Fortier
- Division of Surgical Oncology, Department of Oncology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada; (K.T.); (C.S.); (M.W.); (A.B.-F.)
| | - Lloyd Mack
- Division of Surgical Oncology, Department of Oncology, Cumming School of Medicine, University of Calgary, Calgary, AB T2N1N4, Canada; (K.T.); (C.S.); (M.W.); (A.B.-F.)
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Shastri K, Varma P. Occurrence of a desmoid tumour in a status post appendicectomy: A case report. Int J Surg Case Rep 2024; 125:110652. [PMID: 39586189 PMCID: PMC11625375 DOI: 10.1016/j.ijscr.2024.110652] [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: 09/20/2024] [Revised: 11/19/2024] [Accepted: 11/22/2024] [Indexed: 11/27/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Tumours arising from previous sites of appendicectomy are a rare phenomenon. There have been few documented cases in literature but for the most part, it is a rare occurrence that needs further investigation. CASE PRESENTATION A 20-year-old male presented to our outpatient with a large mass in his right upper abdomen for 2 months and a history of an appendicectomy done in an outside hospital 4 years prior. CLINICAL DISCUSSION After regular investigations, the tumour was resected with adequate margins. On immunohistochemistry, the specimen was said to be of a desmoid fibromatosis. The exact aetiology of the disease cannot be determined unless we get a thorough analysis of the appendix specimen. CONCLUSION This paves the way to the learning curve, which is that we must ensure proper patient follow-ups with relevant histopathological reports to ensure complete and comprehensive care to the patient.
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Affiliation(s)
- Kaushal Shastri
- Department of General Surgery, Kasturba Medical College, Mangalore 575001, India; Manipal Academy of Higher Education, Manipal, Karnataka, India.
| | - Prashant Varma
- Department of General Surgery, Kasturba Medical College, Mangalore 575001, India; Manipal Academy of Higher Education, Manipal, Karnataka, India
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Dhunnoo V, Stevens R, Kinshuck A. Spindle Cell Carcinoma of the Larynx Arising From Aggressive Fibromatosis. Cureus 2024; 16:e74717. [PMID: 39734944 PMCID: PMC11682534 DOI: 10.7759/cureus.74717] [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
Aggressive fibromatosis is a rare, benign proliferative disease with unknown aetiology and high recurrence rate. To date, there are only eight reported cases affecting the larynx. Four were managed with total laryngectomy, whilst spontaneous regression happened in one case. Spindle cell carcinoma is a rare but highly aggressive biphasic tumour which often arises in the head and neck. Diagnosed with laryngeal fibromatosis more than 10 years ago, our patient had undergone six trans-oral laser debulking procedures. On this occasion, he presented with a two-week history of severe worsening dyspnoea and hoarseness of voice, requiring urgent debulking. Final pathology results revealed spindle cell carcinoma of the larynx. In this report, we describe the case of laryngeal fibromatosis which progressed to spindle cell carcinoma. To our knowledge, no such case has been described previously. This case highlights an important complication of laryngeal fibromatosis and emphasises the importance of regular follow-ups.
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Affiliation(s)
- Vedika Dhunnoo
- Otolaryngology - Head and Neck Surgery, Liverpool University Foundation Trust, Liverpool, GBR
| | - Richard Stevens
- Otolaryngology - Head and Neck Surgery, Liverpool University Foundation Trust, Liverpool, GBR
| | - Andrew Kinshuck
- Otolaryngology - Head and Neck Surgery, Aintree University Hospital, Liverpool, GBR
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5
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Bartholomew AJ, Rhodin KE, Noteware L, Moris D, Kanu E, Masoud S, Howell TC, Burner D, Kim CY, Nussbaum DP, Zani S, Lidsky ME, Allen PJ, Riedel RF, Blazer DG. Evolution of Initial Treatment for Desmoid Tumors. Ann Surg Oncol 2024; 31:7943-7949. [PMID: 39133446 DOI: 10.1245/s10434-024-15938-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: 05/13/2024] [Accepted: 07/16/2024] [Indexed: 08/13/2024]
Abstract
INTRODUCTION Desmoid tumors (DTs) are rare, fibroblastic cell proliferations that can exhibit locally aggressive behavior but lack metastatic potential. Initial management has traditionally involved upfront resection; however, contemporary guidelines and expert panels have increasingly advocated for prioritizing active surveillance strategies. METHODS A single-institution, retrospective chart review identified all patients diagnosed with a primary DT at any site from 2007 to 2020. The primary outcome was the initial management strategy over time. Secondary outcomes included treatment-free survival (TFS) and time to treatment (TTT) for those undergoing active surveillance, as well as recurrence-free survival (RFS) and time to recurrence for those undergoing resection. RESULTS Overall, 103 patients were included, with 68% female and a median follow-up of 44 months [24-74]. The most common tumor locations included the abdominal wall (27%), intra-abdominal/mesenteric (25%), chest wall (19%), and extremity (10%). Initial management included resection (60%), systemic therapy (20%), active surveillance (18%), and cryoablation (2%). Rates of surgical resection significantly decreased (p < 0.001) over time, from 69.6% prior to 2018 to 29.2% after 2018. For those treated with upfront resection, 5-year RFS was 41.2%, and for patients undergoing initial active surveillance, TFS was 66.7% at 2 years, with a median TTT of 4 months [4-10]. CONCLUSIONS This single-institution cohort at a tertiary medical center spanning over a decade demonstrates the transition to active surveillance for initial management of DTs, and highlights salient metrics in the era of surveillance. This trend mirrors recommended treatment strategies by expert panels and consensus guidelines.
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Affiliation(s)
- Alex J Bartholomew
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA.
| | - Kristen E Rhodin
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Laura Noteware
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Dimitrios Moris
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Elishama Kanu
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Sabran Masoud
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - T Clark Howell
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Danielle Burner
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Charles Y Kim
- Department of Radiology, Duke University School of Medicine, Durham, NC, USA
| | - Daniel P Nussbaum
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Sabino Zani
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Michael E Lidsky
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Peter J Allen
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
| | - Richard F Riedel
- Department of Medicine, Duke University School of Medicine, Durham, NC, USA
| | - Dan G Blazer
- Department of Surgery, Duke University School of Medicine, Durham, NC, USA
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Tuan NHN, Hai TD, Minh LN, Than TLH, Tu PD, Bao NVT, Van Khoa L, Dang TN, Van Phuoc L. Transarterial doxorubicin-eluting beads embolization for the treatment of desmoid fibromatosis in the pubic region. Radiol Case Rep 2024; 19:4335-4340. [PMID: 39170777 PMCID: PMC11338108 DOI: 10.1016/j.radcr.2024.06.086] [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: 04/09/2024] [Revised: 06/18/2024] [Accepted: 06/30/2024] [Indexed: 08/23/2024] Open
Abstract
Desmoid fibromatosis (DFs) is rare, low-grade neoplasm. Although it poses no risk of metastasis, DFs exhibits a range of clinical manifestations characterized by local infiltrative growth tendencies and a propensity for recurrence. Despite its nonmalignant nature, DFs can be highly debilitating and occasionally life-threatening, causing severe pain and functional limitations. Traditionally, surgery served as the conventional primary treatment approach; nevertheless, a recent shift in paradigm towards a more conservative management has emerged, accompanied by efforts to standardize the strategy among clinicians. Systemic doxorubicin has been demonstrated to be effective in treating DFs; however, it carries potential risks of adverse effects on the cardiovascular, digestive, and hematologic systems. The novel intravascular intervention employing drug-eluting beads loaded with doxorubicin represents an effective treatment for DFs, optimizing drug delivery to the target lesion and reducing systemic toxicity. In this article, we present a rare case of DFs in the right pubic region treated with transarterial doxorubicin-eluting beads embolization.
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Affiliation(s)
| | - Tran Duc Hai
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
| | - Le Nhat Minh
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
| | | | - Pham Dang Tu
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
| | | | - Le Van Khoa
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
| | - Thai Ngoc Dang
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
| | - Le Van Phuoc
- Department of radiology, Cho Ray Hospital, Ho Chi Minh City, Viet Nam
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Almjersah A, Olaisheh H, Salloum R, Alshehabi Z, Almjersah E. Incidental discovery of unilateral hydronephrosis unveiling psoas major desmoid-type fibromatosis in a 24-year-old male: A case report with a 5-year follow-up. Medicine (Baltimore) 2024; 103:e39042. [PMID: 39058836 PMCID: PMC11272233 DOI: 10.1097/md.0000000000039042] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/13/2024] [Accepted: 07/02/2024] [Indexed: 07/28/2024] Open
Abstract
RATIONALE Desmoid-type fibromatosis (DTF), also known as aggressive fibromatosis, is a rare neoplasm originating from the fascial or musculoaponeurotic tissues. While benign and characterized by slow growth, it exhibits local aggressiveness and lacks specific clinical characteristics. However, in a considerable percentage of patients, it could be asymptomatic and discovered by accident during routine clinical examinations. Only a few cases of DTF arising from the psoas major muscle have been reported in the medical literature. PATIENT CONCERNS A 24-year-old male, asymptomatic and without significant personal or family medical history, was diagnosed with grade 2 hydronephrosis by abdominal ultrasonography during a routine physical examination. This diagnosis was made 15 days after undergoing uncomplicated open-heart surgery to repair an atrial septal defect. DIAGNOSIS Intravenous pyelogram revealed hydronephrosis with dilation of the pelvicalyceal system. Ureteroscopy ruled out any intrinsic lesions of the ureter. Contrast-enhanced computed tomography identified a 3.5 × 2 × 5.2 cm mass in the retroperitoneum, closely associated with the psoas muscle and enveloping the ureter adjacent to the iliac artery. Postoperative pathological analysis confirmed a definitive diagnosis of sporadic DTF. INTERVENTIONS The patient underwent exploratory abdominal surgery, during which the tumor was resected without any intraoperative complications. RESULTS After close monitoring over a 5-year follow-up period, which included periodic physical examinations, magnetic resonance imaging, and ultrasonography, no local recurrence was detected. LESSONS Achieving an accurate preoperative diagnosis presents a challenge in cases involving retroperitoneal tumors originating from the psoas major muscle and encasing the ureter. However, the insertion of a double J stent is deemed a crucial step in the surgical process, facilitating the dissection and isolation of the ureter from the tumor while preserving kidney function.
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Affiliation(s)
- Abdulrahman Almjersah
- Faculty of Medicine, Cancer Research Center, Tishreen University, Latakia, Syria
- Syrian Medical Research Group, Damascus, Syria
| | - Habib Olaisheh
- Faculty of Medicine, Cancer Research Center, Tishreen University, Latakia, Syria
- Syrian Medical Research Group, Damascus, Syria
| | - Rabab Salloum
- Department of Pathology, Cancer Research Center, Tishreen University, Latakia, Syria
| | - Zuheir Alshehabi
- Department of Pathology, Cancer Research Center, Tishreen University, Latakia, Syria
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Ahn JH, Lee J, Park C, Beom SH, Kim SH, Lee YH, Yun KH, Kim JE, Baek W, Han YD, Kim SK, Ryu HJ, Jung I, Lee J, Yoon HI, Kim HS. Clinical Activity of TGF-β Inhibitor Vactosertib in Combination with Imatinib in Desmoid Tumors: A Multicenter Phase Ib/II Study. Clin Cancer Res 2024; 30:1457-1465. [PMID: 38363333 DOI: 10.1158/1078-0432.ccr-23-2823] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2023] [Revised: 11/30/2023] [Accepted: 02/14/2024] [Indexed: 02/17/2024]
Abstract
PURPOSE The study was to determine the activity and safety of the TGF-β inhibitor vactosertib in combination with imatinib in patients with desmoid tumors. PATIENTS AND METHODS In this investigator-initiated, open-label, multicenter, phase Ib/II trial, patients with desmoid tumors not amenable to locoregional therapies (surgery and/or radiotherapy) or with disease progression following at least one treatment were enrolled. Participants were administered 400 mg imatinib daily in combination with vactosertib (5 days on and 2 days off, twice a day) every 28 days. In phase Ib, the vactosertib dose was set at 100 mg (level -1) and 200 mg (level 1) to determine the recommended phase II dose (RP2D). Phase II assessed the efficacy, with the primary endpoint being progression-free rate (PFR) at 16 weeks. RESULTS No dose-limiting toxicities were observed during phase Ib; therefore RP2D was defined at doses of 400 mg imatinib daily in combination with 200 mg vactosertib. Of the 27 patients evaluated, 7 (25.9%) achieved a confirmed partial response and 19 (70.4%) were stable. The PFR at 16 weeks and 1 year were 96.3% and 81.0%, respectively. Most toxicities were mild to moderate myalgia (n = 10, 37%), anemia (n = 10, 37%), and nausea (n = 9, 33.3%). Common grade 3 to 4 toxicities included neutropenia (n = 6, 22.2%) and anemia (n = 5, 18.5%). CONCLUSIONS The vactosertib and imatinib combination was well tolerated, with promising clinical activity in patients with progressive, locally advanced desmoid tumors. This is the first study investigating a novel target agent, a TGF-β inhibitor, in this rare and difficult-to-treat desmoid tumor.
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Affiliation(s)
- Jin-Hee Ahn
- Department of Oncology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea
| | - Jeeyun Lee
- Division of Hematology-Oncology, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
| | - Changhee Park
- Department of Internal Medicine, Seoul National University Hospital, Seoul, Republic of Korea
| | - Seung-Hoon Beom
- Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Seung Hyun Kim
- Department of Orthopedic Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Young Han Lee
- Department of Radiology, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Kum-Hee Yun
- Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Jeung Eun Kim
- Department of Oncology, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea
| | - Wooyeol Baek
- Department of Plastic Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Yoon Dae Han
- Department of Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Sang Kyum Kim
- Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Hyang Joo Ryu
- Department of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Inkyung Jung
- Division of Biostatistics, Department of Biomedical Systems Informatics, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - JooHee Lee
- Department of Orthopedic Surgery, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Hong In Yoon
- Department of Radiation Oncology, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Republic of Korea
| | - Hyo Song Kim
- Division of Medical Oncology, Department of Internal Medicine, Yonsei Cancer Center, Yonsei University College of Medicine, Seoul, Republic of Korea
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Najem S, Bekkouche S, Benslimane A, Naciri S, Inrhaouen H, El Ghissassi I, Boutayeb S, Mrabti H, Errihani H. Unraveling the Mystery of Desmoid Tumors: Insights From a Moroccan Tertiary Center. Cureus 2024; 16:e57768. [PMID: 38586227 PMCID: PMC10999233 DOI: 10.7759/cureus.57768] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 04/04/2024] [Indexed: 04/09/2024] Open
Abstract
Desmoid tumors (DTs) are rare, aggressive malignancies developing from clonal fibroblastic proliferation originating from soft tissues. Despite their low metastatic potential, their invasiveness towards neighboring organs and a high recurrence rate contribute significantly to morbidity and mortality, thereby impacting the quality of life of patients. Several therapeutic options are available, but standardized protocols are lacking. In this study, we reviewed 14 cases of DT retrospectively over a period of 15 years, from September 2008 to December 2023. The most prevalent tumor locations were in the extremities, and the majority of patients were female. We identified risk factors in two patients, those being surgical trauma and familial adenomatous polyposis (FAP). Half of the patients underwent surgery for DT, and two received salvage radiotherapy. Systemic therapy was used in the first and second lines and comprised of chemotherapy, endocrine therapy, and non-steroidal anti-inflammatory drugs (NSAI). Active surveillance was proposed in three patients. This is the first retrospective study to assess the characteristics of DT in Moroccan patients in a tertiary care setting. It aims to shed light on the challenges faced in treating these rare tumors in the context of a lack of therapeutic standardization.
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Affiliation(s)
- Salma Najem
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Soukaina Bekkouche
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Amine Benslimane
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Sarah Naciri
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Hanane Inrhaouen
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Ibrahim El Ghissassi
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Saber Boutayeb
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Hind Mrabti
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
| | - Hassan Errihani
- Department of Medical Oncology, National Institute of Oncology, Faculty of Medicine, Mohammed V Faculty, Rabat, MAR
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Narvaez JA, Bernabeu D, Muntaner L, Gomez F, Martel J, Castellano MDM, García-Marcos R, Britel R, Oyagüez I, Tejado N, Ortiz-Cruz E. Economic evaluation of percutaneous cryoablation vs conventional surgery in extra-abdominal desmoid tumours in the Spanish healthcare system. Insights Imaging 2024; 15:1. [PMID: 38185710 PMCID: PMC10772037 DOI: 10.1186/s13244-023-01580-w] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2023] [Accepted: 11/25/2023] [Indexed: 01/09/2024] Open
Abstract
BACKGROUND Desmoid tumours (DTs) or deep fibromatosis are benign soft-tissue tumours, sometimes locally aggressive, requiring intervention on some cases. Surgery has been the gold standard, but new less invasive techniques such as percutaneous cryoablation have proved their effectiveness, reducing health resources and complications. The study aimed to compare the total cost of percutaneous cryoablation and conventional surgery for patients with extra-abdominal and/or abdominal wall DTs, candidates for local ablative treatment in Spain. METHODS A cost-analysis model was developed. An expert panel provided data about resource consumption for the percutaneous cryoablation technique and validated the epidemiology used for target population estimation. Unitary resources cost (€ 2022) derived from local cost databases. A retrospective analysis of 54 surgical cases in 3 Spanish hospitals was performed to estimate the cost of conventional surgery based on the cost of the Diagnosis-Related group (DRG) codes identified on this patient sample, weighted by each DRG proportion. The total cost for each alternative included intervention cost and complications cost, considering debridement required in 4.5% of cases with percutaneous cryoablation and minor surgery for surgical site infection in 18.0% for conventional surgery. RESULTS The total cost for percutaneous cryoablation (€ 5774.78/patient-year) was lower than the total cost for conventional surgery (€ 6780.98/patient-year), yielding cost savings up to € 80,002 in 1 year for the entire cohort of 80 patients with DTs eligible for intervention estimated in Spain. One-way sensitivity analyses confirmed the results' robustness. CONCLUSION Percutaneous cryoablation versus conventional surgery would yield cost savings for the management of DT patients in Spain. CRITICAL RELEVANCE STATEMENT This manuscript provides insight into the economic impact derived from the savings related to the use of percutaneous cryoablation for desmoid-type tumours from the perspective of the Spanish National Healthcare System, providing useful information for the health decision-making process. KEY POINTS • Desmoid tumours are locally aggressive and may require local therapy. • Percutaneous cryoablation procedure is less invasive than the conventional surgery. • Cost comparison shows savings associated to percutaneous cryoablation use.
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Affiliation(s)
- José Antonio Narvaez
- Musculoskeletal Radiology, Hospital Universitario de Bellvitge-IDIBELL, Barcelona, Spain
- Faculty of Medicine and Health Sciences, Universitat de Barcelona, Barcelona, Spain
| | - Daniel Bernabeu
- Musculoskeletal Radiology, Hospital Universitario La Paz, Madrid, Spain
| | - Lorenzo Muntaner
- Hospital Son Espases, Radiología Intervencionista, Mallorca, Spain
| | - Fernando Gomez
- Hospital Sant Joan de Deu, Radiología Intervencionista, Barcelona, Spain
| | - José Martel
- Departamento de Radiología Musculoesqueletica, Hospital Universitario Fundación Alcorcón, Madrid, Spain
| | | | - Raúl García-Marcos
- , Radiología Intervencionista, Hospital Universitario La Fe, Valencia, Spain
| | - Reda Britel
- Hospital Son Espases, Radiología Intervencionista, Mallorca, Spain
| | - Itziar Oyagüez
- Pharmacoeconomics & Outcomes Research Iberia (PORIB), Madrid, Spain.
| | - Nerea Tejado
- Pharmacoeconomics & Outcomes Research Iberia (PORIB), Madrid, Spain
| | - Eduardo Ortiz-Cruz
- Cirugía Ortopédica Oncológica, Hospital Universitario La Paz, Madrid, Spain
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11
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Abufara AA, Alsalah QA, Jabari AY, Hammouri AG, Najajreh M. A 10-year-old male with chest wall Desmoid tumor - a rare tumor with unusual presentation. J Surg Case Rep 2024; 2024:rjae007. [PMID: 38576542 PMCID: PMC10993290 DOI: 10.1093/jscr/rjae007] [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: 12/23/2023] [Accepted: 01/05/2024] [Indexed: 04/06/2024] Open
Abstract
Desmoid fibromatosis (DF) is a connective tissue tumor that grows aggressively in musculoaponeurotic tissues. It has an annual incidence rate of 2-4/million and is commonly seen in individuals aged 15 to 60 years, with female predominance. While it can occur in any body part, it is commonly found in the extremities, abdominal wall, and abdominal mesentery. But it rarely develops in the chest wall. The cause of the tumor is unknown. However, trauma to the tumor site has been identified in 25% of known cases. The primary treatment is surgical resection. Local recurrence after surgical excision is common. Our study highlights the case of a 12-year-old male patient diagnosed with a desmoid tumor on the chest wall 2 years ago, at the age of 10 years, after mild trauma. The tumor was successfully managed with surgery without radiotherapy treatment and no recurrence was observed in the last 2 years.
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Affiliation(s)
- Arein A Abufara
- Faculty of Medicine, Palestine Polytechnic University, Hebron 9020000, Palestine
| | - Qusai A Alsalah
- Faculty of Medicine, Palestine Polytechnic University, Hebron 9020000, Palestine
| | - Anwar Yousef Jabari
- Faculty of Medicine, Palestine Polytechnic University, Hebron 9020000, Palestine
| | - Ahmad G Hammouri
- Radiology Department, Al-Ahli Hospital, Hebron 9020000, Palestine
| | - Mohammad Najajreh
- Faculty of Medicine, Palestine Polytechnic University, Hebron 9020000, Palestine
- Huda Al Masri Pediatric Cancer Department, Beit Jala Governmental Hospital, Bethlehem 9992100, Palestine
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12
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Kyun PJ, Kim DH, Choi CI, Kim KB, Park YM. Laparoscopic gastric wedge resection and spleen-preserving distal pancreatectomy performed on a 6-cm gastric desmoid tumour with pancreatic invasion. J Minim Access Surg 2023; 19:443-446. [PMID: 36695241 PMCID: PMC10449048 DOI: 10.4103/jmas.jmas_177_22] [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: 06/23/2022] [Revised: 08/21/2022] [Accepted: 09/27/2022] [Indexed: 01/22/2023] Open
Abstract
Desmoid tumours are rare tumours originating from fibroblasts, and are characterised by local infiltration and no metastasis. When complete resection is possible, surgical resection is considered a first-line treatment. In the case of large desmoid tumours, it is mainly performed by laparotomy, not laparoscopy. We report a case of a 43-year-old female patient presenting with a hypodense mass of approximately 5 cm in the posterior wall of the gastric antrum on computed tomography. There was no history of familial adenomatous polyposis, trauma or abdominal surgery. The patient underwent laparoscopic gastric wedge resection and spleen-preserving distal pancreatectomy without peri-operative complications. Pathological analysis revealed a desmoid tumour, which originated from the stomach and invaded the pancreas. Despite the large size and the locally infiltrative characteristics of these tumours, laparoscopic surgery can be an optimal treatment option due to its advantages.
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Affiliation(s)
- Park Jae Kyun
- Department of Surgery, College of Medicine, Pusan National University Hospital, Busan, Korea
| | - Dae Hwan Kim
- Department of Surgery, College of Medicine, Pusan National University Hospital, Busan, Korea
| | - Chang In Choi
- Department of Surgery, College of Medicine, Pusan National University Hospital, Busan, Korea
| | - Kyung Bin Kim
- Department of Pathology, College of Medicine, Pusan National University Hospital, Busan, Republic of Korea
| | - Young Mok Park
- Department of Surgery, College of Medicine, Pusan National University Hospital, Busan, Korea
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13
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Kose SI, Singh S, Garg A, Manchanda A, Singh R. Ultrasound and computed tomography in the evaluation of mesenteric lesions: A pictorial review. SA J Radiol 2023; 27:2595. [PMID: 37292421 PMCID: PMC10244954 DOI: 10.4102/sajr.v27i1.2595] [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: 11/22/2022] [Accepted: 03/22/2023] [Indexed: 06/10/2023] Open
Abstract
The mesentery is a broad fan-shaped fold of peritoneum that suspends the loops of small intestine from the posterior abdominal wall. Although primary neoplasms arising in the mesentery are rare, the mesentery is a major avenue for the dissemination of tumours, which can spread through hematogenous, lymphatic, direct or peritoneal seeding. Imaging helps in the diagnosis of these tumours and aids in directing appropriate treatment by assessing their size, extent and relationship with adjacent structures. The aim of this article is to describe the spectrum of imaging findings of the various mesenteric lesions using ultrasound and CT. Contribution Evaluation of the mesentery is often neglected during routine ultrasound (US) because of inadequate training and unfamiliarity with the common US features encountered with mesenteric disease. CT plays an essential role in the diagnosis of mesenteric disease. Knowledge of imaging characteristics of various mesenteric lesions helps in timely diagnosis and management.
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Affiliation(s)
- Snehal I Kose
- Department of Radiodiagnosis, Maulana Azad Medical College, Delhi, India
| | - Sapna Singh
- Department of Radiodiagnosis, Maulana Azad Medical College, Delhi, India
| | - Anju Garg
- Department of Radiodiagnosis, Maulana Azad Medical College, Delhi, India
| | - Alpana Manchanda
- Department of Radiodiagnosis, Maulana Azad Medical College, Delhi, India
| | - Rajdeep Singh
- Department of Surgery, Maulana Azad Medical College, Delhi, India
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14
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Figueredo C, Schiano T. A Review of the Clinical Presentation, Outcomes, and Treatments of Patients Having Desmoid Tumors. GASTRO HEP ADVANCES 2023; 2:588-600. [PMID: 39132032 PMCID: PMC11308132 DOI: 10.1016/j.gastha.2023.01.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/21/2022] [Accepted: 01/13/2023] [Indexed: 08/13/2024]
Abstract
Desmoid tumors (DTs) are deep fibroblastic neoplasms that arise from musculo-aponeurotic stromal elements. DTs may result in significant morbidity by infiltrating vital anatomic structures. Their mortality is often due to the local aggressiveness, most commonly when intra-abdominal in location. Some indolent DTs can be observed expectantly; infiltrative tumors require an aggressive and multidisciplinary approach and are offered conservative therapies such as nonsteroidal anti-inflammatory drugs or antiestrogens when surgery is not feasible. Comparably, chemotherapy is considered for those cases not amenable to surgery or radiation. Bowel resection and at times intestinal transplantation may be necessary. However, DTs may recur postsurgery making long-term management of these patients. Herein, we review the genetics, clinical presentations, outcomes, and treatments of DTs.
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Affiliation(s)
- Carlos Figueredo
- Department of Gastroenterology and Hepatology, Montefiore Medical Center/Albert Einstein College of Medicine, New York, New York
| | - Thomas Schiano
- Recanati-Miller Transplantation Institute, The Mount Sinai Medical Center, New York, New York
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15
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Tee SW, Richards A, Tan YH, Jeyabalan D, Durairaj G. Desmoid fibromatosis infiltrating left adrenal gland and kidney. J Surg Case Rep 2022; 2022:rjac585. [PMID: 36578629 PMCID: PMC9792179 DOI: 10.1093/jscr/rjac585] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2022] [Accepted: 11/26/2022] [Indexed: 12/27/2022] Open
Abstract
Desmoid fibromatosis is a rare, benign, locally aggressive fibroblastic proliferation that may occur in almost any anatomical location. Due to its rarity and unpredictable clinical course, there has not been a standard guideline of treatment. We encountered a case of desmoid fibromatosis in our centre. A young lady previously fit and well was referred for a symptomatic, rapidly growing left sided abdominal mass. Otherwise, she denied any bowel related symptoms or constitutional manifestation. Imaging demonstrated a large well-defined lobulated solid-cystic mass extending from vertebral level T10 to L5, measuring 10.5 cm × 15 cm × 23 cm. The mass was in close proximity with the left adrenal gland, left kidney, pancreas and spleen. Ultrasound guided biopsy interpreted it as a fibroblastic or myelofibroblastic tumour, favouring desmoid fibromatosis. Surgery was then performed where the mass was removed along with the left adrenal gland and kidney. Post-operative care was complicated with pulmonary embolism, hospital-acquired pneumonia and pancreatitis.
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Affiliation(s)
- Sui Wu Tee
- Correspondence address. Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia. E-mail:
| | - Avisha Richards
- Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia
| | - Yi Han Tan
- Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia
| | - Dhinisya Jeyabalan
- Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia
| | - Gunaseelan Durairaj
- Department of General and Vascular Surgery, Serdang Hospital, Selangor, 43000 Kajang, Malaysia
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Hajri M, Talbi G, Ferjaoui W, Atallah A, Ben Slama S, Mestiri H, Bayar R. Huge mesenteric desmoid-type fibromatosis with unusual presentation: A case report. Ann Med Surg (Lond) 2022; 78:103741. [PMID: 35600202 PMCID: PMC9118479 DOI: 10.1016/j.amsu.2022.103741] [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: 03/19/2022] [Revised: 05/05/2022] [Accepted: 05/08/2022] [Indexed: 11/26/2022] Open
Abstract
Introduction Desmoid-type fibromatosis, also known as desmoid tumors, are rare fibroblastic neoplasms that account for less than 3% of all soft tissue tumors. Although they are benign neoplasms without metastatic potential, they are known to be locally aggressive and may invade adjacent structures leading to fatal complications. Case presentation We describe the case of a 26-year-old woman who presenting with the clinical picture of acute peritonitis. Emergency surgery was performed and a large poorly-circumscribed heterogeneous tumor was found, occupying the jejunum mesentery and infiltrating the jejunal wall causing its perforation into the abdominal cavity. En bloc resection of the tumor and the involved jejunum was performed. Histology and immunohistochemistry confirmed it to be mesenteric desmoid-type fibromatosis. The postoperative course was uneventful and the patient had no evidence of recurrence 18 months after tumor resection. Conclusions Mesenteric desmoid-type fibromatosis is a rare condition with insidious growth and locally aggressive behavior. Serious complications such as bowel perforation are rare but possible, as shown in our presentation. Complete surgical resection is the first-line treatment bur high recurrence rates remain problematic.
Desmoid-typefibromatosis are rare fibroblastic neoplasms that account for less than 3% of all soft tissue tumors. We reported a rare case of diffuse peritonitis due to sporadic mesenteric desmoid-type fibromatosis with aggressive proliferation and consecutive intestinal perforation. Only a few similar cases have been reported in the literature. This case report an unusual presentation of mesenteric desmoid.
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17
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Wang J, Li H, Wang H, Li Q, Bai X, Lv H, Nie C, Chen B, Xu W, Tu S, Chen X. Combination of Anlotinib and Celecoxib for the Treatment of Abdominal Desmoid Tumor: A Case Report and Literature Review. Front Oncol 2022; 11:830672. [PMID: 35096630 PMCID: PMC8792388 DOI: 10.3389/fonc.2021.830672] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/07/2021] [Accepted: 12/20/2021] [Indexed: 11/20/2022] Open
Abstract
Desmoid tumor is a rare disease, which is histologically characterized by local invasion, monoclonality, and fibroblast proliferation; and clinically characterized by a variable and often unpredictable course. The treatment of desmoid tumor is mainly surgical resection, but the recurrence rate is high. In recent years, a variety of treatment methods, including endocrine therapy, surgery, radiotherapy, chemotherapy, non-steroidal anti-inflammatory drugs, targeted drugs, interferon and more, have been used and achieved certain curative effects. In addition, in view of the inertia characteristics of desmoid tumor, observation is also a first-line scheme recommended by multiple guidelines. In the past, the research progress of targeted therapy for desmoid tumor is relatively slow and the curative effect is limited. Thus, targeted therapy is usually used as a remedial treatment after the failure of other conventional treatment methods. However, in recent years, with the rapid progress in the basic research of targeted therapy, some new targeted drugs are increasingly used for the clinical treatment of desmoid tumor and have achieved good results. Herein, we described a patient with aggressive fibromatosis in the abdominal cavity. Following a combined treatment using anlotinib and celecoxib, the patient achieved a partial response with mild toxicity. Simultaneously, the patient’s pain symptoms completely disappeared. This case indicates that the combination of anlotinib and NSAIDs could be an effective treatment for desmoid tumor.
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Affiliation(s)
- Jianzheng Wang
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Hongle Li
- Department of Molecular Pathology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Hui Wang
- Department of Endoscope Center, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Qingli Li
- Department of Oncology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Xuanye Bai
- Department of Pathology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, Henan, China
| | - Huifang Lv
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Caiyun Nie
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Beibei Chen
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Weifeng Xu
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
| | - Shuiping Tu
- Department of Oncology, Renji Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China
| | - Xiaobing Chen
- Department of Medical Oncology, Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, China
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18
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Lahoud J, Fong V, Rajendran S, Tse V. Desmoid fibromatosis involving the ureter: A rare presentation with intraoperative challenges. Urol Case Rep 2021; 40:101883. [PMID: 34712580 PMCID: PMC8528671 DOI: 10.1016/j.eucr.2021.101883] [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: 09/24/2021] [Revised: 10/06/2021] [Accepted: 10/11/2021] [Indexed: 10/26/2022] Open
Abstract
Following failed retrograde and antegrade ureteric stenting, a 35-year-old male patient underwent an elective boari flap for marked proximal hydroureteronephrosis due to a periureteric mass in the right iliac fossa. Intraoperative vascular surgical assistance was required for control of arterial bleeding due to friable vessel wall. Histopathology demonstrated desmoid fibromatosis.
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Affiliation(s)
- John Lahoud
- Department of Urology, Concord Hospital, Sydney, Australia
| | - Voon Fong
- Department of Urology, Concord Hospital, Sydney, Australia
| | | | - Vincent Tse
- Department of Urology, Concord Hospital, Sydney, Australia
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19
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Mabrouk MY, Bouzayan L, Malki S, Jabi R, Bennani A, Bouziane M. Desmoid tumor of the anterolateral abdominal wall: A rare case report. Ann Med Surg (Lond) 2021; 70:102804. [PMID: 34691412 PMCID: PMC8519763 DOI: 10.1016/j.amsu.2021.102804] [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: 05/26/2021] [Revised: 08/31/2021] [Accepted: 09/03/2021] [Indexed: 11/22/2022] Open
Abstract
INTRODUCTION AND IMPORTANCE Desmoid tumors of the abdominal wall are rare fibroblastic proliferative tissue, included in the group of soft tissue tumors, not metastasizing but locally aggressive as an infiltrating tumor and a source of recurrence. CASE PRESENTATION This case report describes a rare case of desmoid tumor of the anterolateral abdominal wall presented with a large mass of the left flank and iliac fossa. The diagnostic was suspected radiologically following an abdominopelvic computed tomography (CT) and magnetic resonance imaging (MRI). An exploratory laparotomy found a large mass infiltrating the left rectus abdominis muscle, a part of the flat left abdominal muscles, and the left iliac crest. The patient underwent a total resection of the mass involving the left rectus muscle with autoplasty by a pedicled flap of the left LATA fascia with the placement of a bifacial mesh.Histologic analysis of the operatory specimen confirmed the diagnosis of a desmoid tumor of the abdominal wall.The patient has been discharged from the hospital on the fifth day post-operatory with an uneventful recovery; she was in good health after a one-year follow-up. CLINICAL DISCUSSION Desmoid tumors of the abdomen are very rare. Although this tumor is histologically benign, it has the potential of invading vital structures and has a high rate of local recurrence.Histology staining confirms the diagnosis, surgery is the gold standard in the management of this pathology. CONCLUSION We highlight the importance of radical surgical excision to avoid desmoid tumor complications and to minimize the recurrence risk.
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Affiliation(s)
- Mohamed Yassine Mabrouk
- Department of General Surgery, Mohamed VI University Hospital, Oujda, Morocco
- Faculty of Medicine and Pharmacy, Laboratory of Anatomy, Microsurgery and Surgery Experimental and Medical Simulation (LAMCESM), Mohammed 1st University, Oujda, Morocco
| | - Laila Bouzayan
- Department of General Surgery, Mohamed VI University Hospital, Oujda, Morocco
- Faculty of Medicine and Pharmacy, Laboratory of Anatomy, Microsurgery and Surgery Experimental and Medical Simulation (LAMCESM), Mohammed 1st University, Oujda, Morocco
| | - Samia Malki
- Department of Pathology, Mohammed VI University Hospital, Oujda, Morocco
| | - Rachid Jabi
- Department of General Surgery, Mohamed VI University Hospital, Oujda, Morocco
- Faculty of Medicine and Pharmacy, Laboratory of Anatomy, Microsurgery and Surgery Experimental and Medical Simulation (LAMCESM), Mohammed 1st University, Oujda, Morocco
| | - Amal Bennani
- Department of Pathology, Mohammed VI University Hospital, Oujda, Morocco
| | - Mohammed Bouziane
- Department of General Surgery, Mohamed VI University Hospital, Oujda, Morocco
- Faculty of Medicine and Pharmacy, Laboratory of Anatomy, Microsurgery and Surgery Experimental and Medical Simulation (LAMCESM), Mohammed 1st University, Oujda, Morocco
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20
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Tumor and tumorlike conditions of the pleura and juxtapleural region: review of imaging findings. Insights Imaging 2021; 12:97. [PMID: 34236561 PMCID: PMC8266978 DOI: 10.1186/s13244-021-01038-x] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/01/2021] [Accepted: 06/11/2021] [Indexed: 01/09/2023] Open
Abstract
Pleural lesions form a diagnostic challenge for the radiologist. Whereas lesions can be initially detected on chest radiographs, CT and MRI imaging are the imaging modalities of choice for further characterization. In a number of cases, imaging findings can be relatively specific. In general unfortunately, imaging findings are rather aspecific. Evolution and extrathoracic imaging findings are important clues toward the diagnosis.
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Abstract
OBJECTIVES Fibromatosis is a rare tumor with a prevalence of 2-4 per million per year. Its occur-rence in the thyroid is very rare and to the best of our knowledge only five cases have been reported in the literature.Subject and Results. In this case report, we present a thirty-seven-year-old man without any significant past medical history, who presented with an anterior neck swelling and occasional cough for four months before admission. Sonographic-guided fine needle aspiration cytology studies reported a follicular neoplasm. Microscopic and immunohistochemical evaluation of the thyroidectomy specimen showed thyroid fibromatosis. The patient had no symptoms after surgery and no signs of recurrence after 2 years of follow-up.
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22
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Auloge P, Garnon J, Robinson JM, Thenint MA, Koch G, Caudrelier J, Weiss J, Cazzato RL, Kurtz JE, Gangi A. Percutaneous cryoablation for advanced and refractory extra-abdominal desmoid tumors. Int J Clin Oncol 2021; 26:1147-1158. [PMID: 33709291 DOI: 10.1007/s10147-021-01887-y] [Citation(s) in RCA: 17] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2020] [Accepted: 02/11/2021] [Indexed: 01/21/2023]
Abstract
PURPOSE To assess efficacy and safety of percutaneous cryoablation (CA) for advanced and refractory extra-abdominal desmoid tumors. MATERIALS AND METHODS This retrospective study reviewed 30 consecutive patients with symptomatic desmoid tumors evolving after "wait and watch" periods, and despite medical treatment, treated by CA between 2007 and 2019. Progression free survival (PFS), objective response rate, pain reduction (decreased of visual analogic scale pain (VAS)[Formula: see text] 3 or disappearance of pain), total volume lesion (TVL) and complications were documented. Kaplan Meier method was used to outline PFS. Paired sample t test was used to compare volume of tumors before treatment and at 1 and 3 year. RESULTS With a median follow-up of 18.5 months (range 6-93 months, interquartile range (IQR): 12-55), the PFS was 85.1% at 1 year and 77.3% at 3 years. Objective response was obtained for 80% of patients with a complete response for 43% patients. Pain reduction was obtained for 96.7% (95% confidence interval (95% CI): 90.3, 100) of patients. Median volume of desmoid tumor before treatment was 124.1cm3 (range 2-1727cm3, IQR: 54-338cm3). Median change of TLV after ablation was 66.6% (95% CI: 37.2, 72.3; p = 0.002) at 1 year and 76.4% (95% CI: 59.1, 89.8; p = 0.002) at 3 year. Adverse events rate was 36.6%, the most common was edema and temporary increase of pain in the days following CA. Four patients experienced a major complication (13.3%): 2 skin necrosis, 1 infection and 1 brachial plexopathy. CONCLUSION CA is an effective treatment for advanced and refractory extra-abdominal desmoid tumor, that induces durable responses.
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Affiliation(s)
- Pierre Auloge
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France.
| | - Julien Garnon
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | - Joey Marie Robinson
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | | | - Guillaume Koch
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | - Jean Caudrelier
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | - Julia Weiss
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | - Roberto Luigi Cazzato
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
| | - Jean Emmanuel Kurtz
- Service d'Oncologie, Hôpitaux Universitaires de Strasbourg, Strasbourg, France
| | - Afshin Gangi
- Service d'Imagerie Interventionnelle, Hôpitaux Universitaires de Strasbourg, 3 rue des Alisiers, 67100, Strasbourg, France
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23
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Guo L, Wang X, Xu B, Lang R, Hu B. Prognostic significance of CTNNB1 mutation in recurrence of sporadic desmoid tumors. Future Oncol 2021; 17:435-442. [PMID: 33397129 DOI: 10.2217/fon-2020-0544] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2020] [Accepted: 10/07/2020] [Indexed: 11/21/2022] Open
Abstract
Aim: Desmoid tumor (DT) is a rare, locally aggressive benign neoplasm with a high recurrence rate. The majority of sporadic DTs are associated with mutations in CTNNB1, but whether CTNNB1 mutations are associated with the risk of DT recurrence remains unclear. The goal of this meta-analysis was to evaluate the association between CTNNB1 mutation and recurrence in surgically treated DT patients. Methods: PubMed, Embase and Cochrane library were systematically searched. The outcome of interest was the risk of recurrence. The number of patients with CTNNB1 mutation and the number of recurrences they developed were recorded and compared. The quality of these studies was assessed using the Newcastle-Ottawa Quality Assessment Scale. Odds ratios and variances were calculated and pooled. Results: A total of eight studies were identified including 637 patients. S45F-mutated DTs were more likely to recur compared with wild type, T41A and other mutated DTs. However, there were no statistically significant differences in the rate of recurrence between wild type and T41A mutation or other mutation. Conclusions: Among CTNNB1 mutations, the mutation S45F is a high-risk factor for recurrence of DT and may be a predictive marker for the recurrence of sporadic DT.
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Affiliation(s)
- Lifang Guo
- Department of Thoracic Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020, China
| | - Xin Wang
- Pharmacy Department, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China
| | - Benshan Xu
- Pharmacy Department, Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China
| | - Ren Lang
- Department of Hepatobiliary Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020, China
| | - Bin Hu
- Department of Thoracic Surgery, Beijing Chaoyang Hospital, Capital Medical University, Beijing, 100020, China
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Huned D, Tiwari RV, Lee LS. Unusual finding after treatment for testicular cancer. BMJ Case Rep 2021; 14:14/1/e233701. [PMID: 33419745 PMCID: PMC7799132 DOI: 10.1136/bcr-2019-233701] [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: 01/11/2023] Open
Abstract
In the context of a post-treatment testicular germ cell tumour, an abdominal lesion found on surveillance CT studies led to a differential diagnosis, including recurrent germ cell tumour. We report the case of a 48-year-old man who was noted to have a new interval soft tissue lesion on a surveillance CT scan, 5 years after initial orchidectomy and chemotherapy. Excision of this lesion and histopathological review revealed an intra-abdominal desmoid.
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25
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Martin D, Labgaa I, Petermann D, Di Mare L. Small bowel obstruction caused by a fast-growing desmoid tumor. Clin Case Rep 2020; 8:2318-2319. [PMID: 33235792 PMCID: PMC7669366 DOI: 10.1002/ccr3.3162] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/27/2020] [Accepted: 06/13/2020] [Indexed: 11/12/2022] Open
Abstract
Desmoid tumors are rare tumors which can cause intestinal obstructions. Surgical wide excision is currently the treatment of choice, with the goal of achieving free resection margins.
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Affiliation(s)
- David Martin
- Department of Visceral SurgeryUniversity Hospital CHUV and University of LausanneLausanneSwitzerland
| | - Ismail Labgaa
- Department of Visceral SurgeryUniversity Hospital CHUV and University of LausanneLausanneSwitzerland
- Department of General and Visceral SurgeryEHC HospitalMorgesSwitzerland
| | - David Petermann
- Department of General and Visceral SurgeryEHC HospitalMorgesSwitzerland
| | - Luca Di Mare
- Department of General and Visceral SurgeryEHC HospitalMorgesSwitzerland
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Rosa F, Martinetti C, Piscopo F, Buccicardi D, Schettini D, Neumaier CE, Gandolfo N, Grazioli L, Gastaldo A. Multimodality imaging features of desmoid tumors: a head-to-toe spectrum. Insights Imaging 2020; 11:103. [PMID: 32986198 PMCID: PMC7520866 DOI: 10.1186/s13244-020-00908-0] [Citation(s) in RCA: 30] [Impact Index Per Article: 6.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2020] [Accepted: 09/03/2020] [Indexed: 12/11/2022] Open
Abstract
Desmoid tumors (DTs) are a rare and biologically heterogeneous group of locally aggressive fibroblastic neoplasm: their biological behavior spectrum ranges from indolent to aggressive tumors. DTs are classified as intra-abdominal, extra-abdominal, and within the abdominal wall lesions.It is well known that abdominal and extra-abdominal DTs are associated with familial adenomatous polyposis (FAP) and Gardner syndrome. Possible risk factors are prior trauma/surgery, pregnancy, and oral contraceptives.There was a real revolution in the management of DT: from aggressive first-line approach (surgery and radiation therapy) to a more conservative one (systemic treatment and "wait-and-see policy").In these clinical settings, radiologists play an important role for assessing lesion resectability, evaluating recurrence, monitoring the biological behavior if an expectant management is chosen, and assessing response to systemic treatment as well as to radiation therapy.Awareness of common locations, risk factors, and imaging features is fundamental for a correct diagnosis and an adequate patient management.
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Affiliation(s)
- F Rosa
- Diagnostic Imaging Department, San Paolo Hospital-ASL 2, via Genova, 30, Savona, Italy.
| | - C Martinetti
- Department of Health Sciences (DISSAL), University of Genova, via A. Pastore 1, 16132, Genova, Italy
| | - F Piscopo
- Diagnostic Imaging Department, San Paolo Hospital-ASL 2, via Genova, 30, Savona, Italy
| | - D Buccicardi
- Diagnostic Imaging Department, San Paolo Hospital-ASL 2, via Genova, 30, Savona, Italy
| | - D Schettini
- Diagnostic Imaging Department, Villa Scassi Hospital-ASL 3, Corso Scassi 1, Genova, Italy
| | - C E Neumaier
- Diagnostic Imaging and Senology Unit, Policlinico San Martino, Largo R. Benzi 10, 16132, Genova, Italy
| | - N Gandolfo
- Diagnostic Imaging Department, Villa Scassi Hospital-ASL 3, Corso Scassi 1, Genova, Italy
| | - L Grazioli
- ASST "Spedali Civili", P.le Spedali Civili 1, 25123, Brescia, Italy
| | - A Gastaldo
- Diagnostic Imaging Department, San Paolo Hospital-ASL 2, via Genova, 30, Savona, Italy
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27
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Mujtaba B, Call C, Rowland F, Spear RP, Amini B, Valenzuela R, Nassar S. Desmoid fibromatosis following surgical resection of spinal meningioma. Radiol Case Rep 2020; 15:697-701. [PMID: 32280401 PMCID: PMC7139138 DOI: 10.1016/j.radcr.2020.02.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2020] [Revised: 02/05/2020] [Accepted: 02/13/2020] [Indexed: 11/21/2022] Open
Abstract
A 42-year-old female patient with a history of cervicothoracic junction meningioma World Health Organization grade I, resected in early 2011, was admitted to the hospital with intractable headache and lower extremity weakness. Magnetic resonance imaging (MRI) showed an epidural mass compressing the spinal cord at the prior surgical site, which was interpreted as recurrent meningioma. Following surgical resection, histopathological analysis revealed desmoid fibromatosis (desmoid tumor). In retrospect, dynamic contrast-enhanced magnetic resonance imaging performed prior to surgery should have allowed for prospective exclusion of meningioma as the recurrent mass and suggested an alternative diagnosis such as post-traumatic desmoid fibromatosis or the need for biopsy to confirm diagnosis.
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Bajaj G, Tirumani H, Whisman MK, Raichandani S, Ram R, Jambhekar K, Gardner JM, Pandey T. Comprehensive Review of Abdominopelvic Mesenchymal Tumors with Radiologic Pathologic Correlation and Update on Current Treatment Guidelines - Part 1. Semin Ultrasound CT MR 2020; 41:222-238. [PMID: 32446433 DOI: 10.1053/j.sult.2020.01.002] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Soft-tissue sarcomas are a diverse group of rare mesenchymal malignancies accounting for only 1% of all solid adult malignancies. These have been categorized in 12 broad groups by the World Health Organization (WHO) with their recent update in 2013. Majority of them lack specific imaging features serving as imaging conundrums for a radiologist. These are often large masses at presentation as they are asymptomatic or cause vague clinical symptoms. These tumors are challenging for surgeons as well as they find it difficult to achieve complete resection because of complex intra-abdominal anatomy and their close relationship with critical structures. Often, a multidisciplinary approach is required to decide on the most appropriate management for these complex cases so as to provide optimal patient care. Knowledge of the WHO classification, pathologic features and available treatment options will help the radiologist make a meaningful contribution in multidisciplinary discussions of such cases and overall patient care. Liposarcoma (well-differentiated and dedifferentiated liposarcomas), leiomyosarcoma, and gastrointestinal stromal tumor are the 3 most common primary intra-abdominal sarcomas. In part 1 of this article, general features of soft tissue sarcomas and some of the common tumors from WHO category 1-4 found in abdomen and pelvis are discussed. Part 2 will focus on common tumors from remainder of the WHO categories.
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Affiliation(s)
- Gitanjali Bajaj
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR.
| | - Harika Tirumani
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR
| | - Michella K Whisman
- Department of Pathology and Clinical Laboratories, University of Michigan, Ann Arbor, MI
| | - Surbhi Raichandani
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR
| | - Roopa Ram
- Department of Radiology, University of Arkansas for Medical Sciences, Little Rock, AR
| | - Kedar Jambhekar
- Department of Radiology and Orthopedics, University of Arkansas for Medical Sciences, Little Rock, AR
| | - Jerad M Gardner
- Department of Pathology and Dermatology, University of Arkansas for Medical Sciences, Little Rock, AR
| | - Tarun Pandey
- Department of Radiology and Orthopedics, University of Arkansas for Medical Sciences, Little Rock, AR
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29
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de Bruyns A, Li H, MacNeil A, Simmons C, Clarkson P, Goddard K, Munk PL, Hart JJ, Holloway C, Truong P, Feng X. Evolving Practice Patterns Over Two Decades (1993-2013) in the Management of Desmoid-type Fibromatosis in British Columbia. Clin Oncol (R Coll Radiol) 2019; 32:e102-e110. [PMID: 31685376 DOI: 10.1016/j.clon.2019.10.005] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2019] [Revised: 09/19/2019] [Accepted: 10/04/2019] [Indexed: 01/24/2023]
Abstract
AIMS Due to the rarity and varied natural history of desmoid-type fibromatosis, evidence-based treatment standards for this disease remain lacking. This study evaluated outcomes in patients with desmoid-type fibromatosis managed at a Canadian institution over two decades. MATERIALS AND METHODS Records of 227 patients with desmoid-type fibromatosis referred from 1990 to 2013 were retrospectively reviewed to investigate management strategies including active surveillance, surgery, radiation therapy, cryoablation, and systemic therapy, including tamoxifen and chemotherapy. RESULTS Thirty-two per cent of cases were men, median age 40 years, median tumour size 5.4 cm. Initial treatments were surgery (79%), tamoxifen (13%), radiation therapy (5.0%), chemotherapy (1.8%) and cryoablation (1.2%). Active surveillance was used upfront in 26% of cases, most after 2005. At a median follow-up of 77 months, one patient died of disease, 13 died of unrelated causes and the remainder were alive with no evidence of disease (56%), stable/responding disease (33%) or progressive disease (4%). The recurrence rate was 25% after upfront surgery. Response rates and disease control rates were 40% and 76% for active surveillance; 68% and 96% for radiation therapy; 31% and 67% for tamoxifen; and 53% and 80% for chemotherapy. On univariable analysis, factors associated with a higher recurrence after initial surgery were young age (P = 0.012), male gender (P = 0.012) and extremity location (P = 0.005). On multivariable analysis, only young age was significantly associated with recurrence risk (P = 0.010). CONCLUSIONS Active surveillance was associated with spontaneous regression and long-term disease control consistent with other studies. Primary radiation therapy appeared to provide a similar response and disease control compared with systemic treatments and may be a viable option for patients who are not candidates for surgery or active surveillance.
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Affiliation(s)
- A de Bruyns
- Faculty of Medicine, Island Medical Program, University of British Columbia, Victoria, British Columbia, Canada
| | - H Li
- Department of Mathematics and Statistics, University of Calgary, Calgary, Alberta, Canada
| | - A MacNeil
- Department of Surgery, Vancouver General Hospital, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - C Simmons
- Department of Medical Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - P Clarkson
- Department of Surgery, Vancouver General Hospital, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - K Goddard
- Department of Radiation Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - P L Munk
- Department of Medical Imaging, Vancouver General Hospital, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - J J Hart
- Department of Medical Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - C Holloway
- Department of Radiation Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - P Truong
- Department of Radiation Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada
| | - X Feng
- Department of Medical Oncology, University of British Columbia, British Columbia Cancer Agency - Vancouver Center, Vancouver, British Columbia, Canada.
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30
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Gondim Teixeira PA, Biouichi H, Abou Arab W, Rios M, Sirveaux F, Hossu G, Blum A. Evidence-based MR imaging follow-up strategy for desmoid-type fibromatosis. Eur Radiol 2019; 30:895-902. [PMID: 31468156 DOI: 10.1007/s00330-019-06404-4] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/21/2019] [Revised: 07/04/2019] [Accepted: 07/29/2019] [Indexed: 10/26/2022]
Abstract
OBJECTIVES To propose a follow-up strategy for desmoid-type fibromatosis (DF) based on tumor growth behavior and the signal on T2-weighted MRI. METHODS We retrospectively reviewed 296 MRI studies of 34 patients with histologically proven DF. In each study, tumor volume and T2 signal relatively normal striated muscle were assessed. Volume variation and monthly growth rates were analyzed to determine lesion growth behavior (progressing versus stable/regressing lesions). Growth behavior was correlated with T2 signal, tumor location, β-catenin status, treatment strategy, and follow-up duration. Interobserver variability of volume measurements and interobserver measurement variation ratio were assessed. RESULTS There were 25 women and 9 men with a mean age of 39.9 ± 19 (4-73) years. Mean follow-up time in the patients included was 55 ± 41 (12-148) months. In progressing lesions, the mean average monthly growth ratio was 10.9 ± 9.2 (1.1-42.5) %. Interobserver variability of volume measurements was excellent (ICC = 0.96). Mean interobserver measurement variation ratio was 20.4 ± 23.6%. The only factor correlated with tumor growth behavior was T2 signal ratio (p < 0.0001). Seventeen out of 34 (50%) patients presented a signal change over the threshold of 1 during follow-up. There were five occurrences of secondary growth after a period of stability with a mean delay until growth of 38.2 ± 44.2 (17-116) months. CONCLUSION DF growth rate was quantitatively assessed. A threshold for volume variation detection was established. DF growth behavior was significantly related to T2 signal. An evidence-based follow-up strategy is proposed. KEY POINTS • In progressing desmoid fibromatosis, the mean average monthly growth ratio was 10.9 ± 9.2%. • Lesions with muscle/tumor T2 signal ratios lower than 1 tended to be stable or regress over time. • Given the interobserver measurement variability and MRI in-plane spatial resolution, a variation higher than 42.6% in tumor volume is required to confirm punctual progression.
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Affiliation(s)
- P A Gondim Teixeira
- Guilloz Imaging Department, Central Hospital, Regional University Hospital Center of Nancy (CHRU-Nancy), 29 avenue du Maréchal de Lattre de Tassigny, 54035, Nancy, France.
| | - H Biouichi
- Guilloz Imaging Department, Central Hospital, Regional University Hospital Center of Nancy (CHRU-Nancy), 29 avenue du Maréchal de Lattre de Tassigny, 54035, Nancy, France
| | - W Abou Arab
- Guilloz Imaging Department, Central Hospital, Regional University Hospital Center of Nancy (CHRU-Nancy), 29 avenue du Maréchal de Lattre de Tassigny, 54035, Nancy, France
| | - M Rios
- Lorraine Cancer Institute, Vandoeuvre-lès-Nancy, France
| | - F Sirveaux
- Emile Gallé Surgical Center, Regional University Hospital Center of Nancy, Nancy, France
| | - G Hossu
- Inserm, IADI, Université de Lorraine, Nancy, France
| | - A Blum
- Guilloz Imaging Department, Central Hospital, Regional University Hospital Center of Nancy (CHRU-Nancy), 29 avenue du Maréchal de Lattre de Tassigny, 54035, Nancy, France
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Muneer M, Badran S, Zahid R, Abdelmageed A, AlDulaimi MM. Recurrent Desmoid Tumor with Intra-Abdominal Extension After Abdominoplasty: A Rare Presentation. AMERICAN JOURNAL OF CASE REPORTS 2019; 20:953-956. [PMID: 31270310 PMCID: PMC6621933 DOI: 10.12659/ajcr.916227] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
BACKGROUND Desmoid tumors are fibrous neoplasms that originate from the musculoaponeurotic structures in the body. Abdominal wall desmoid tumors are rare, but they can be locally aggressive, with high incidence of recurrence. These tumors are more common in young, fertile women. They frequently occur during or after pregnancy. CASE REPORT We present the case of a 63-year-old post-menopausal woman with a desmoid tumor of the anterior abdominal wall. She had no relevant family history. During abdominoplasty, an incidental mass was excised and biopsied, and was identified as a desmoid tumor with free margins. One year later, the patient presented with vague abdominal discomfort and feeling of heaviness. An incision was made through the previous abdominoplasty scar to maintain the aesthetic outcome. A large mass, arising from the abdominal wall and extending intra-abdominally, was excised and was determined to be a recurrent desmoid tumor. CONCLUSIONS Recurrent anterior abdominal wall desmoid tumors in post-menopausal women are rare and locally aggressive, with a high risk of recurrence. During abdominal wall repair in abdominoplasty, desmoid tumor filaments might seed deep intra-abdominally. Therefore, it is necessary to take adequate safe margins before abdominal wall repair. Post-operatively, surgeons should keep a high index of suspicion for tumor recurrence.
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Affiliation(s)
- Mohammed Muneer
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar.,Department of Surgery, Weill Cornel Medical College, Doha, Qatar
| | - Saif Badran
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar
| | - Rehan Zahid
- Department of Plastic Surgery, Hamad Medical Corporation, Doha, Qatar
| | - Amal Abdelmageed
- Department of Health and Life Sciences, Hamad bin Khalifa University, Doha, Qatar
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32
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Zhou L, Xu H, Zhou J, Dong L, Zhang P, Yang X, Wang C. Nuclear TFE3 expression is a diagnostic marker for Desmoid-type fibromatosis. Diagn Pathol 2019; 14:34. [PMID: 31043173 PMCID: PMC6495536 DOI: 10.1186/s13000-019-0814-4] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/12/2018] [Accepted: 04/17/2019] [Indexed: 12/22/2022] Open
Abstract
BACKGROUND Desmoid-type fibromatosis (DTF) is a lesion characterized by clonal proliferation of myofibroblasts, which exhibits an infiltrative growth pattern. It is necessary for them to be distinguished from other fibroblastic and myofibroblastic lesions as well as spindle cell tumors. Altered Wnt signaling can act as a defining characteristic of DTF, with nuclear β-catenin serving as a diagnostic marker for. Transcription factor E3 (TFE3) has been linked to Wnt pathway activation and regulation, and may add value to the diagnosis of DTF. The present study, therefore, sought to assess whether TFE3 is a specific diagnostic marker for DTF. METHODS Nuclear TFE3 and β-catenin staining was performed on a wide range of tumor types such as DTF (n = 46), nodular fasciitis (n = 14), neurofibroma (n = 5), dermatofibrosarcoma protuberans (n = 5), gastrointestinal stromal tumor (n = 10), sclerosing epithelioid fibrosarcoma (n = 2), synovial sarcoma (n = 5), leiomyoma (n = 3) and cutaneous scar tissue (n = 4) using an immunohistochemical approach. In addition, the clinicopathological features and localization of these tumors were summarized. FISH assay was carried out to examine Xp11.2 translocations/TFE3 gene fusions. Statistical difference between immunohistochemical expression of TFE3 and β-catenin was analyzed. RESULTS The expression of nuclear TFE3 protein was found in 43 (93.5%) DTF tissue samples, ranging from moderate to intense expression levels. The distribution rates of TFE3 positivity in nodular fasciitis, gastrointestinal stromal tumor, leiomyoma and scar tissue samples were 42.9, 40, 25 and 33%, respectively. All studied samples of neurofibroma, synovial sarcoma, sclerosing epithelioid fibrosarcoma and dermatofibrosarcoma protuberans were negative for TFE3. CONCLUSIONS This study reveal that TFE3 has a potential to serve as a diagnostic marker capable of assisting in the differential diagnosis of DTF and other spindle cell lesions.
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Affiliation(s)
- Luting Zhou
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Haimin Xu
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Jun Zhou
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Lei Dong
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Peipei Zhang
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Xiaoqun Yang
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China
| | - Chaofu Wang
- Department of Pathology, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, 200025, China.
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Timbergen MJ, Janssen ML, Verhoef C, Grünhagen DJ, Chibon F, Smid M, Sleijfer S, Wiemer EA. Wnt targets genes are not differentially expressed in desmoid tumors bearing different activating β-catenin mutations. EUROPEAN JOURNAL OF SURGICAL ONCOLOGY 2019; 45:691-698. [PMID: 30528042 DOI: 10.1016/j.ejso.2018.09.019] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2018] [Revised: 09/17/2018] [Accepted: 09/24/2018] [Indexed: 11/21/2022]
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34
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Primary desmoid tumor in renal transplant graft site: First case report. Urol Case Rep 2019; 23:44-45. [PMID: 30581752 PMCID: PMC6297126 DOI: 10.1016/j.eucr.2018.12.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/18/2018] [Revised: 12/04/2018] [Accepted: 12/05/2018] [Indexed: 11/24/2022] Open
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35
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Ji H, Zhu W, Zhao B, Shi J, Wei Q, Sun B, Chen Q. A giant mesenteric fibromatosis involving the muscular layer of the colon wall: A case report. Medicine (Baltimore) 2019; 98:e14015. [PMID: 30608449 PMCID: PMC6344171 DOI: 10.1097/md.0000000000014015] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/26/2022] Open
Abstract
RATIONALE Mesenteric fibromatosis (MF) is a rare tumor whose biological behavior is intermediate between benign fibrous neoplasms and fibrosarcomas, and the characteristic of these tumors are local aggressive lesions which is prone to local recurrence but non-metastasizing. The common symptom is abdominal distention or painless mass. We report a case of giant MF in abdominal cavity with abdominal distention as the main symptom. PATIENT CONCERNS A 26-year-old male presented with 2-month history of abdominal distention, lack of appetite, and symptoms grew progressively more debilitating with time. DIAGNOSES This patient underwent a contrast-enhanced computed tomography scan which showed a giant (37 × 25 × 13 cm), inhomogeneous enhancing, well-defined, and soft tissue density mass in abdominal cavity, possibly arising in mesocolon, which suggested a high possibility of MF. The postoperative pathology showed that the tumor cells to be positive for β-catenin, vimentin, negative for CD34, CD117, DOG-1, S-100, Desmin, which confirmed the diagnosis of MF. INTERVENTIONS Exploratory laparotomy was performed, which revealed a large mass involving the transverse colon wall, the root of mesocolon, and encasing the middle colic vessels and the 1st branch of jejunal arteries. The complete surgical resection was performed and the mass weighted 10 kilograms (kg). OUTCOMES The patient recovered uneventfully and was discharged 9 days after surgery. Three-month, 6-month, 12-month and 18-month on follow-up after surgery, showed no evidence of recurrence. LESSONS The MF is a very rare tumor, especially a giant tumor (10 kg) involving the muscular layer of colon wall. In addition, treatment of giant MF still remains a challenge. We consider that surgical resection with negative margins is the goal but not at the expense of damaging the function of vital organs. Specific measures should be considered based on the individual patient in order to relieve symptoms and improve quality of life.
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Affiliation(s)
- Haibin Ji
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Wentao Zhu
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Baolei Zhao
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Jian Shi
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Qiang Wei
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Baofang Sun
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
| | - Qiangpu Chen
- Department of Hepatobiliary Surgery, Clinical Nutrition Support Center, Binzhou Medical University Hospital
- Clinical Nutrition and Metabolism Key Laboratory of Shandong Province, Binzhou, Shandong, China
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36
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Elkhouly AG, Cervelli V, Sanese G, Pompeo E. Hump-like giant desmoid tumor of the chest: a postresectional reconstruction challenge. AME Case Rep 2018; 1:6. [PMID: 30263993 DOI: 10.21037/acr.2017.11.01] [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: 10/13/2017] [Accepted: 10/31/2017] [Indexed: 11/06/2022]
Abstract
Desmoid tumors (DT) are rare neoplasms with unknown etiology arising from musculoaponeurotic structures. Chest wall localization is uncommon and has been associated with high recurrence rate unless radical resection with negative margins is carried out. Postresectional reconstruction can be challenging in presence of giant lesions and might require adoption of complex reconstruction methods including use of well vascularized muscle flaps. We present a case of giant hump-like recurrent chest wall DT, which was radically resected following placement of multiple subcutaneous silicon tissue expanders, to gain redundant skin, which eventually allowed in conjunction with two transposition, cutaneous-adipose flaps, harvested from the upper gluteal region, an optimal reconstruction of the large postresectional defect.
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Affiliation(s)
- Ahmed G Elkhouly
- Department of Cardiothoracic Surgery, Faculty of Medicine, Tanta University, Tanta, Egypt
| | - Valerio Cervelli
- Department of Plastic and Reconstructive Surgery, Policlinico Tor Vergata University, Rome, Italy
| | - Giuseppe Sanese
- Department of Plastic and Reconstructive Surgery, Policlinico Tor Vergata University, Rome, Italy
| | - Eugenio Pompeo
- Department of Thoracic Surgery, Policlinico Tor Vergata University, Rome, Italy
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37
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Libertini M, Mitra I, van der Graaf WTA, Miah AB, Judson I, Jones RL, Thomas K, Moskovic E, Szucs Z, Benson C, Messiou C. Aggressive fibromatosis response to tamoxifen: lack of correlation between MRI and symptomatic response. Clin Sarcoma Res 2018; 8:13. [PMID: 29785261 PMCID: PMC5950191 DOI: 10.1186/s13569-018-0100-3] [Citation(s) in RCA: 30] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2018] [Accepted: 04/04/2018] [Indexed: 01/22/2023] Open
Abstract
Background One of the commonly used systemic agents for the treatment of aggressive fibromatosis is the anti-oestrogen drug tamoxifen. However, data on efficacy and optimum methods of response assessment are limited, consisting mainly of small case series and reports. Methods A retrospective database was used to identify consecutive patients diagnosed with aggressive fibromatosis (AF) and treated with tamoxifen plus/minus non-steroidal anti-inflammatory drugs at our tertiary referral centre between 2007 and 2014. MRI and symptom changes were recorded. Results Thirty-two patients (13 male 19 female, median age 41 years) were included. Median duration of treatment with tamoxifen was 316 days. Of 9 patients with progressive disease by RECIST 1.1 (28%): 4 patients experienced worsening symptoms; 3 patients had improved symptoms and 2 had no change in symptoms. Of 22 patients with stable disease (69%): 11 had no change in symptoms; 6 had improved symptoms and 5 patients had worsening symptoms. One patient achieved a partial response with improved symptoms. Conclusions No relationship was identified between symptomatic benefit and response by RECIST 1.1 on MRI. Prospective studies in AF should incorporate endpoints focusing on patient symptoms.
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Affiliation(s)
- M Libertini
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK
| | - I Mitra
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,2Department of Radiology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK
| | - W T A van der Graaf
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,3Institute of Cancer Research, Sutton, UK
| | - A B Miah
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,3Institute of Cancer Research, Sutton, UK
| | - I Judson
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,3Institute of Cancer Research, Sutton, UK
| | - R L Jones
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,3Institute of Cancer Research, Sutton, UK
| | - K Thomas
- 2Department of Radiology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK
| | - E Moskovic
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,3Institute of Cancer Research, Sutton, UK
| | - Z Szucs
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK
| | - C Benson
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK
| | - C Messiou
- 1Sarcoma Unit, Royal Marsden NHS Foundation Trust, London, UK.,2Department of Radiology, The Royal Marsden NHS Foundation Trust, 203 Fulham Road, London, SW3 6JJ UK.,3Institute of Cancer Research, Sutton, UK
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Jambhekar A, Robinson S, Zafarani P, Rucinski J, Gudavalli P. Intestinal obstruction caused by desmoid tumours: a review of the literature. JRSM Open 2018; 9:2054270418763340. [PMID: 29760935 PMCID: PMC5946608 DOI: 10.1177/2054270418763340] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/10/2023] Open
Abstract
Intraabdominal desmoid tumours are rare and can cause intestinal obstruction. Based on the review of the literature, surgical resection with negative margins and adjuvant chemotherapy is the optimal strategy for treatment of this pathology.
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Affiliation(s)
- Amani Jambhekar
- Department of Surgery, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY 11215, USA
| | - Shawn Robinson
- Department of Surgery, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY 11215, USA
| | - Parvin Zafarani
- Department of Surgery, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY 11215, USA
| | - James Rucinski
- Department of Surgery, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY 11215, USA
| | - Prasad Gudavalli
- Department of Surgery, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY 11215, USA
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Ong Q, Wong J, Sinha S, Kejriwal N. Desmoid fibromatosis of the chest wall. Respirol Case Rep 2018; 6:e00310. [PMID: 29507725 PMCID: PMC5830061 DOI: 10.1002/rcr2.310] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/10/2017] [Revised: 01/27/2018] [Accepted: 02/05/2018] [Indexed: 11/09/2022] Open
Abstract
We report a case of desmoid fibromatosis of the chest wall. A 70-year-old woman was referred to our hospital with right shoulder blade pain and paresthesia over the right upper breast. Chest X-ray and computed tomography demonstrated a 5 cm right apical mass in the chest. Biopsy of the mass demonstrated features of desmoid fibromatosis. The patient subsequently underwent surgical resection of the mass and received adjuvant radiation therapy for microscopic positive margins. In conclusion, although desmoid tumour of the chest is rare, it is worth considering in the differential diagnoses of chest wall tumours.
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Affiliation(s)
- QiHao Ong
- Department of Cardiothoracic SurgeryWaikato District Health BoardHamiltonNew Zealand
| | - Janice Wong
- Department of Respiratory MedicineWaikato District Health BoardHamiltonNew Zealand
| | - Sanjay Sinha
- Department of PathologyWaikato District Health BoardHamiltonNew Zealand
| | - Nand Kejriwal
- Department of Cardiothoracic SurgeryWaikato District Health BoardHamiltonNew Zealand
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Diagnostic imaging and CEUS findings in a rare case of Desmoid-type fibromatosis. A case report. J Ultrasound 2018; 21:253-257. [PMID: 29564660 DOI: 10.1007/s40477-018-0291-8] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/04/2017] [Accepted: 02/13/2018] [Indexed: 12/27/2022] Open
Abstract
Desmoid-type fibromatosis (DF), also known as aggressive fibromatosis, is a locally aggressive benign fibroblastic neoplasm that can infiltrate or recur but cannot metastasize. It is rare, with an estimated annual incidence of two to four new cases per million people. Most DFs occur sporadically, but it may also be associated with the hereditary syndrome familial adenomatous polyposis. Treatment is necessary when the disease is symptomatic, especially in case of compression of critical structures. When possible, surgical resection is the treatment of choice; however, recurrence is common. Due to the high rate of recurrence, imaging plays an important role not only in diagnosis, but also in the management of DF. Although there are a number of studies describing CT and MRI findings of DF, there is no description of contrast-enhanced ultrasound findings.
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Desmoid Tumor and Silicone Breast Implant Surgery: Is There Really a Connection? A Literature Review. Aesthetic Plast Surg 2018; 42:59-63. [PMID: 28842766 DOI: 10.1007/s00266-017-0948-2] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/19/2017] [Accepted: 07/17/2017] [Indexed: 12/30/2022]
Abstract
BACKGROUND Desmoid tumors are borderline tumors of the connective tissue, arising in the musculo-aponeurotic stromal elements. A desmoid tumor (DT) has an infiltrative and locally aggressive growth pattern and usually does not metastasize; however, it has a high recurrence and complication rate. DT located in the breast (BDT) represents a rare extra-abdominal form. Recently, the presence of breast silicone implants was suggested by several researchers as a risk factor for developing BDT. OBJECTIVES The goal of this review is to investigate the possible correlation between BDT and breast implant surgery. METHODS We conducted a literature review of BDT-reported cases, associated with breast implant surgery. RESULTS The search revealed 36 cases of BDT associated with silicone breast implants. CONCLUSIONS Based on the reviewed data, the incidence of BDT following breast implant surgery is lower than BDT in the general population. At the moment, a possible association between breast implants and the development of breast desmoid tumors cannot be unequivocally confirmed. A world registry with accurate documentation of each case of BDT associated with breast implant surgery should be performed for future investigation. LEVEL OF EVIDENCE II This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to Table of Contents or the online Instructions to Authors www.springer.com/00266 .
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42
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Krishnamurthy G, Jha VC, Verma GR. Port site desmoid tumour following laparoscopic cholecystectomy: A case report. J Minim Access Surg 2018; 14:247-249. [PMID: 29319019 PMCID: PMC6001309 DOI: 10.4103/jmas.jmas_209_17] [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] [Indexed: 11/24/2022] Open
Abstract
Desmoid tumours are locally aggressive tumours occurring either spontaneously or in familial conditions. History of trauma is invariably present with surgical trauma being a common cause. Port site desmoid tumours are extremely rare conditions. Inadequate treatment results in high recurrence rate and substantial morbidity. Reconstruction, if required, by the appropriate technique is vital to avoid an incisional hernia. Adjuvant therapy may be useful in large locally advanced or recurrent tumours. We describe a young female with large port site desmoid tumour following laparoscopic cholecystectomy managed with wide local excision and mesh placement.
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Affiliation(s)
- Gautham Krishnamurthy
- Department of General Surgery, Division of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Vijay Chetan Jha
- Department of General Surgery, Division of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
| | - Ganga Ram Verma
- Department of General Surgery, Division of Surgical Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
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Martínez Trufero J, Pajares Bernad I, Torres Ramón I, Hernando Cubero J, Pazo Cid R. Desmoid-Type Fibromatosis: Who, When, and How to Treat. Curr Treat Options Oncol 2017; 18:29. [PMID: 28439797 DOI: 10.1007/s11864-017-0474-0] [Citation(s) in RCA: 70] [Impact Index Per Article: 8.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022]
Abstract
OPINION STATEMENT Desmoid-type fibromatosis is a sarcoma subtype that gathers some singular characteristics, making it a difficult challenge to face in clinical practice. Despite its excellent survival prognosis, these tumors may be unpredictable, ranging from an asymptomatic indolent course to persistent, local, and extended recurrences that significantly impair quality of life. Although surgery was initially considered the first elective treatment, collected published data during the past few years are now pointing to the "wait and see" approach as a reasonable initial strategy because many patients can live a long life with the disease without having symptoms. When symptoms appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) can be useful in improving symptoms and controlling the disease. Because of the low incidence of desmoid-type fibromatosis, there is scarce scientific evidence supporting any specific treatment. Nonetheless, if volumetric responses are needed, chemotherapy may be a reasonable early option. However, if long-term control of disease is desirable, hormonal therapy, NSAIDs, and TKIs are the likely treatments of choice. Recent new findings in the biologic development of these tumors, such as the role of Wnt/β-catenin dependent pathway, have shown that the prognostic information provided by specific CTNNB1 gene mutations and other genetic profiles can lead to better methods of selecting patients as candidates for other approaches. Based on recent research, the Notch pathway inhibition in DF is one of the most promising potential targets to explore. As an orphan disease, it is mandatory that as many patients as possible be included in clinical trials.
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Affiliation(s)
- Javier Martínez Trufero
- Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain.
| | - Isabel Pajares Bernad
- Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain
| | - Irene Torres Ramón
- Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain
| | - Jorge Hernando Cubero
- Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain
| | - Roberto Pazo Cid
- Medical Oncology Department, Hospital Universitario Miguel Servet, Avda Isabel la Católica 1-3, 50009, Zaragoza, Spain
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Skubitz KM. Biology and Treatment of Aggressive Fibromatosis or Desmoid Tumor. Mayo Clin Proc 2017; 92:947-964. [PMID: 28578783 DOI: 10.1016/j.mayocp.2017.02.012] [Citation(s) in RCA: 69] [Impact Index Per Article: 8.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/31/2016] [Revised: 01/24/2017] [Accepted: 02/16/2017] [Indexed: 12/14/2022]
Abstract
Aggressive fibromatosis, also known as desmoid-type fibromatosis (DTF) or desmoid tumor, is an uncommon locally invasive tumor. Because of its low incidence and variable behavior, DTF is often first seen by physicians who are not familiar with it, and recent advances in understanding this disease have led to changes in treatment approaches. The Wnt (β-catenin) pathway appears to play a key role in DTF pathogenesis, and recent studies of DTF biology suggest a possible model of DTF pathogenesis. Histologically, DTF shows a poorly circumscribed proliferation of myofibroblast-like cells with variable collagen deposition, similar to the proliferative phase of wound healing, and DTF has been associated with trauma and pregnancy. Desmoid-type fibromatosis may be a useful model of the tumor stroma in carcinomas as well as other fibrosing diseases such as progressive pulmonary fibrosis. The clinical course of DTF can vary greatly among patients, complicating the determination of the optimal treatment approach. Treatment options include surgery, nonsteroidal anti-inflammatory drugs with or without hormonal manipulation, chemotherapy, radiation therapy, and other forms of local therapy. Many treatments have been used, but these are not without toxicities. Because of the variable nature of the disease and the potential morbidity of treatment, some cases of DTF may do better without treatment; simple observation is often the best initial treatment. This review used a PubMed search from January 1, 1980, through October 31, 2016, using the terms fibromatosis and desmoid and discusses DTF disease characteristics, pathophysiology, and treatment options as well as examines several cases illustrating key points in the biology and treatment of this heterogeneous disease.
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Affiliation(s)
- Keith M Skubitz
- Department of Medicine, University of Minnesota Medical School, Minneapolis.
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45
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Munhoz AM, Marques ADA, Milanez JR, Gemperli R. Chest wall reconstruction following axillary breast augmentation and desmoid tumor resection using capsular flaps and a form-stable silicone implant: A case report, diagnosis and surgical technique. Int J Surg Case Rep 2017; 36:110-115. [PMID: 28554106 PMCID: PMC5447517 DOI: 10.1016/j.ijscr.2017.05.023] [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: 02/11/2017] [Revised: 05/14/2017] [Accepted: 05/15/2017] [Indexed: 01/09/2023] Open
Abstract
Desmoid tumors (DT) are a rare benign lesion, and chest desmoid tumors are much less frequent. A rare case of chest DT is presented and discussed. Chest DT following breast augmentation is very rare and few cases have been described; all have been related to the inframammary approach and located in the implant capsule. The authors describe a chest DT following subfascial axillary breast augmentation and subsequent surgical management and chest wall reconstruction. The case illustrates some aspects that may be useful for thoracic and plastic surgeons and may be used as guidance for surgical management. Introduction Chest desmoid tumors (CDT) are rare lesions characterized by fibroblastic proliferation from the connective tissue. Although CDT have been studied previously, no cases following subfascial transaxillary breast augmentation (TBA) have been described. Presentation of case The authors describe a case of CDT in a 28-year-old woman one year after TBA, which presented as a painful and progressive mass in the lower-inner right breast quadrant. MRI showed a soft-tissue tumor (6 × 3 × 4 cm) that affected the region of the right anterior costal margin, without signs of structural costal invasion. Patient was treated surgically, exposing the right costal-sternal region through an inframammary approach and resecting the CDT. The remaining capsular flap was mobilized into the defect and a form-stable silicone implant was utilized to cover the chest wall defect and achieve an adequate breast contour. The patient is currently in 5th year after chest reconstruction, with satisfactory results. Neither the tumor or the symptoms recurred. Discussion CDT is an uncommon evolution following TBA. Although it is a rare disease, thoracic and plastic surgeons must be alert to avoid misdiagnosis. Defect reconstruction is necessary, mobilizing the capsular flaps and replacing the implants in order to obtain a satisfactory outcome. Conclusion Knowledge of this rare post-operative evolution is crucial, and early surgical intervention is warranted in order to avoid more aggressive treatment. This case report provides general knowledge of CDT, and may be used as guidance for early diagnosis and treatment.
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Affiliation(s)
| | | | - José Ribas Milanez
- Hospital Albert Einstein and Hospital das Clínicas University of São Paulo, Brazil
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Hayashi K, Takamura M, Yokoyama H, Sato Y, Yamagiwa S, Nogami H, Wakai T, Hasegawa G, Terai S. A Mesenteric Desmoid Tumor with Rapid Progression. Intern Med 2017; 56:505-508. [PMID: 28250295 PMCID: PMC5399200 DOI: 10.2169/internalmedicine.56.7320] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/26/2023] Open
Abstract
We herein report the case of a rapidly progressive sporadic mesenteric desmoid tumor (DT). A 62-year-old woman presented with a 4-cm-diameter palpable mass in the left supraumbilical area. The mass showed an ill-defined margin with heterogeneous delayed enhancement on computed tomography and heterogeneous high intensity on T2-weighted magnetic resonance imaging. Sixteen months after the initial observation, the mass had grown in size, reaching 13 cm in diameter. The resected mass was histologically confirmed as a DT of the mesentery. Since DT often has an unpredictable clinical course, clinicians should bear in mind the need for imaging follow-up.
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Affiliation(s)
- Kazunao Hayashi
- Division of Gastroenterology and Hepatology, Niigata University Graduate School of Medical and Dental Sciences, Japan
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Kopric D, Nurkanovic L, Alidzanovic J. Extra-abdominal desmoid tumor: A case report. INTERNATIONAL JOURNAL OF CANCER THERAPY AND ONCOLOGY 2016. [DOI: 10.14319/ijcto.43.3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022] Open
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Saito Y, Hinoi T, Ueno H, Kobayashi H, Konishi T, Ishida F, Yamaguchi T, Inoue Y, Kanemitsu Y, Tomita N, Matsubara N, Komori K, Kotake K, Nagasaka T, Hasegawa H, Koyama M, Ohdan H, Watanabe T, Sugihara K, Ishida H. Risk Factors for the Development of Desmoid Tumor After Colectomy in Patients with Familial Adenomatous Polyposis: Multicenter Retrospective Cohort Study in Japan. Ann Surg Oncol 2016; 23:559-565. [PMID: 27387679 DOI: 10.1245/s10434-016-5380-3] [Citation(s) in RCA: 30] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/10/2016] [Indexed: 12/17/2022]
Abstract
BACKGROUND Desmoid tumor (DT) is the primary cause of death in patients with familial adenomatous polyposis (FAP) after restorative proctocolectomy. This study aimed to identify risk factors for DT in a Japanese population. METHODS Clinical data for 319 patients with FAP undergoing first colectomy from 2000 to 2012 were reviewed retrospectively. RESULTS Two hundred seventy-seven FAP patients were included in this study. Thirty-nine (14.1 %) patients developed DT. Occurrence sites were the intraperitoneal region in 25 (64.1 %) cases, intraperitoneal region and abdominal wall in three (7.7 %), and abdominal wall in nine (23.1 %). The mean period from surgery to DT development was 26.3 months (range 4-120 months). Gender (female vs. male, p = 0.03), age at surgery (>30 vs. ≤30 years, p = 0.02), purpose of surgery (prophylactic vs. cancer excision, p = 0.01), and surgical procedure (proctocolectomy [ileoanal anastomosis (IAA), ileoanal canal anastomosis (IACA), total proctocolectomy (TPC)] vs. total colectomy [ileorectal anastomosis, partial colectomy]; p = 0.03) significantly influenced the estimated cumulative risk of developing DT at 5 years after surgery. Conversely, approach (laparoscopic vs. open, p = 0.17) had no significant effect on the increased risk of DT occurrence. In multivariate analysis, female gender, with a hazard ratio of 2.2 (p = 0.02,) and proctocolectomy (IAA, IACA, TPC), with a hazard ratio of 2.2 (p = 0.03), were independent risk factors for DT incidence after colectomy. CONCLUSIONS Female gender and proctocolectomy (IAA, IACA, TPC) were independent risk factors for developing DT after colectomy in patients with FAP.
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Affiliation(s)
- Yasufumi Saito
- Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Takao Hinoi
- Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan.
| | - Hideki Ueno
- Department of Surgery, National Defense Medical College, Tokorozawa, Saitama, Japan
| | - Hirotoshi Kobayashi
- Center for Minimally Invasive Surgery, Tokyo Medical and Dental University, Bunkyō, Tokyo, Japan
| | - Tsuyoshi Konishi
- Gastroenterological Center, Department of Gastroenterological Surgery, Cancer Institute Hospital, Japanese Foundation for Cancer Research, Tokyo, Japan
| | - Fumio Ishida
- Digestive Disease Center, Showa University Northern Yokohama Hospital, Yokohama, Kanazawa, Japan
| | - Tatsuro Yamaguchi
- Department of Surgery, Tokyo Metropolitan Cancer and Infectious Diseases Center Komagome Hospital, Tokyo, Japan
| | - Yasuhiro Inoue
- Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Tsk, Mie, Japan
| | - Yukihide Kanemitsu
- Division of Colorectal Surgery, National Cancer Center Hospital, Tokyo, Japan
| | - Naohiro Tomita
- Department of Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
| | - Nagahide Matsubara
- Department of Surgery, Hyogo College of Medicine, Nishinomiya, Hyogo, Japan
| | - Koji Komori
- Department of Gastroenterological Surgery, Aichi Cancer Center Hospital, Nagoya, Aichi, Japan
| | - Kenjiro Kotake
- Department of Surgery, Tochigi Cancer Center, Utsunomiya, Tochigi, Japan
| | - Takeshi Nagasaka
- Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
| | | | - Motoi Koyama
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, Hirosaki, Aomori, Japan
| | - Hideki Ohdan
- Department of Gastroenterological and Transplant Surgery Applied Life Sciences, Institute of Biomedical & Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Toshiaki Watanabe
- Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Bunkyō, Tokyo, Japan
| | | | - Hideyuki Ishida
- Department of Digestive Tract and General Surgery, Saitama Medical Center, Saitama Medical University, Saitama, Japan
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Campara Z, Spasic A, Aleksic P, Milev B. An Aggressive Retroperitoneal Fibromatosis. Med Arch 2016; 70:154-7. [PMID: 27147794 PMCID: PMC4851536 DOI: 10.5455/medarh.2016.70.154-157] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/05/2016] [Accepted: 03/15/2016] [Indexed: 11/25/2022] Open
Abstract
Introduction: Aggressive fibromatosis (AF) is a heterogeneous group of mesenchymal tumors that have locally infiltrative growth and a tendency to relapse. The clinical picture is often conditioned by the obstruction of the ureter or small intestine. Diagnosis is based on clinical, radiological and histological parameters. A case report: We report a case of male patient, aged 35 years, with the retroperitoneal fibromatosis. He reported to the physician because of frequent urination with the feeling of pressure and pain. Computed tomography revealed the tumor mass on the front wall of the bladder with diameter of 70mm with signs of infiltration of the musculature of the anterior abdominal wall. Endoscopic transurethral biopsy showed proliferative lesion binders by type of fibromatosis. The tumor was surgically removed in a classical way. The patient feels well and has no recurrence thirty-six months after the operative procedure. Conclusion: The complete tumor resection is the therapeutic choice for the primary tumor as well as for a relapse.
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Affiliation(s)
- Zoran Campara
- Department of Urology, Military Medical Academy, Belgrade, Serbia
| | | | - Predrag Aleksic
- Department of Urology, Military Medical Academy, Belgrade, Serbia
| | - Bosko Milev
- Department of Abdominal Surgery, Military Medical Academy, Belgrade, Serbia
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50
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Rahvar M, Kerstetter J. Cutaneous manifestation of gastrointestinal disease. J Gastrointest Oncol 2016; 7:S44-54. [PMID: 27034812 PMCID: PMC4783618 DOI: 10.3978/j.issn.2078-6891.2015.059] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/27/2015] [Accepted: 03/10/2015] [Indexed: 12/29/2022] Open
Abstract
The gastrointestinal (GI) and cutaneous systems are closely linked in origin. Skin manifestations are frequently seen as a part of different GI syndromes. Gastroenterologists play an important role in recognizing the symptoms, patient workup and arriving at appropriate diagnoses, often in consultation with dermatologists. This review discusses the diseases with both cutaneous and intestinal involvement. Hereditary polyposis GI cancers, hereditary nonpolyposis colorectal cancers (CRCs), hamartomatous disorders, and inflammatory bowel disease (IBD) are reviewed with emphasis on the genetic basis, diagnostic, histologic findings, screening modalities, and therapeutic options.
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Affiliation(s)
- Maral Rahvar
- Department of Pathology and Laboratory Medicine, Loma Linda University Medical Center, Loma Linda, CA, USA
| | - Justin Kerstetter
- Department of Pathology and Laboratory Medicine, Loma Linda University Medical Center, Loma Linda, CA, USA
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