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Yan L, Huang R, Chen S, Chen J, Li J. Imaging findings of primary hepatic leiomyosarcoma: a case report and literature review. Front Oncol 2024; 14:1490717. [PMID: 39640280 PMCID: PMC11617361 DOI: 10.3389/fonc.2024.1490717] [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: 09/03/2024] [Accepted: 10/30/2024] [Indexed: 12/07/2024] Open
Abstract
Primary hepatic leiomyosarcoma (PHLS) is an extremely rare malignant tumor, which is often elusive in early diagnosis due to its rarity and nonspecific clinical and imaging presentations. Herein, we present a case of PHLS in a 66-year-old male and a review of the English literature from January 2000 to December 2023, focusing on the clinical and imaging characteristics of 30 patients with PHLS. The present patient was admitted to our hospital with complaints of abdominal distension, with history of hepatitis B. Tumor markers, including alpha-fetoprotein, carcinoembryonic antigen, and CA 19-9, were within the normal range. A hepatic tumor was incidentally identified during an abdominal ultrasound examination, further evaluated by contrast-enhanced CT and MR scans, which was preliminarily misdiagnosed as hepatocellular carcinoma. The tumor was surgically excised and definitively diagnosed as PHLS, characterized by two distinct areas with varying imaging features on contrast-enhanced CT and MR images. PHLS typically manifests as a well-defined, heterogeneously hypo- or iso-dense mass on CT, with a slightly prolonged T2 signal on MRI, and exhibits gradual enhancement during dynamic contrast-enhanced imaging. We advocate that the possibility of PHLS should be considered when the aforementioned imaging features are observed.
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Affiliation(s)
- Lifen Yan
- Department of Radiology, Guangdong Provincial People′s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China
| | - Runqian Huang
- Department of Radiology, Guangdong Provincial People′s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China
| | - Shuting Chen
- Department of Radiology, Guangdong Provincial People′s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China
| | - Jiawei Chen
- Department of Radiology, People’s Hospital of Yingde City, Yingde, Guangdong, China
| | - Jinglei Li
- Department of Radiology, Guangdong Provincial People′s Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China
- Department of Radiology, Key Laboratory of Molecular Diagnosis & Disease Prevention, Heyuan People's Hospital, Heyuan, China
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Wang B, Chen X, Li R, Ai B, Ye F, Zhao J, Zhang Y, Huang Z, Li Z, Bi X, Zhao H, Cao D, Cai J, Zhou J, Yan T. Comprehensive evaluation of clinical outcomes in hepatic epithelioid hemangioendothelioma subsets: insights from SEER Database and departmental cohort analysis. Front Immunol 2024; 15:1491922. [PMID: 39502705 PMCID: PMC11534872 DOI: 10.3389/fimmu.2024.1491922] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/05/2024] [Accepted: 10/07/2024] [Indexed: 11/08/2024] Open
Abstract
Background Epithelioid hemangioendothelioma (EHE), is an uncommon, intermediate-grade malignant vascular tumor that can manifest in diverse organs, including the liver, lungs, and bones. Given its unique malignancy profile and rarity, there lacks a consensus on a standardized treatment protocol for EHE, particularly for hepatic epithelioid hemangioendothelioma (HEHE). This study aims to elucidate factors influencing the clinical prognosis of EHE by analyzing data from the SEER database, complemented with insights from a departmental cohort of 9 HEHE cases. Through this, we hope to shed light on potential clinical outcomes and therapeutic strategies for HEHE. Methods Using SEER data from 22 registries, we analyzed 313 liver cancer patients with ICD-O-3 9130 and 9133 histology. Twelve variables were examined using Cox regression and mlr3 machine learning. Significant variables were identified and compared. Clinical data, imaging characteristics, and treatment methods of nine patients from our cohort were also presented. Result In univariate and multivariate Cox regression analyses, Age, Sex, Year of diagnosis, Surgery of primary site, Chemotherapy, and Median household income were closely related to survival outcomes. Among the ten survival-related machine learning models, CoxPH, Flexible, Mboost, and Gamboost stood out based on Area Under the Curve(AUC), Decision Curve Analysis(DCA), and Calibration Curve Metrics. In the feature importance analysis of these four selected models, Age and Surgery of primary site were consistently identified as the most critical factors influencing prognosis. Additionally, the clinical data of nine patients from our cohort not only demonstrated unique imaging characteristics of HEHE but also underscored the importance of surgical intervention. Conclusion For patients with resectable HEHE, surgical treatment is currently a highly important therapeutic approach.
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Affiliation(s)
- Bingchen Wang
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xiao Chen
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Rongxuan Li
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Bolun Ai
- Department of Breast Surgical Oncology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Feng Ye
- Department of Radiology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Jianjun Zhao
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yefan Zhang
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhen Huang
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhiyu Li
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Xinyu Bi
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Hong Zhao
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Dayong Cao
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Jianqiang Cai
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Jianguo Zhou
- Department of Hepatobiliary Surgery, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Tao Yan
- Department of Anesthesiology, National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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3
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Biolato M, Miele L, Marrone G, Tarli C, Liguori A, Calia R, Addolorato G, Agnes S, Gasbarrini A, Pompili M, Grieco A. Frequency of and reasons behind non-listing in adult patients referred for liver transplantation: Results from a retrospective study. World J Transplant 2024; 14:92376. [PMID: 38947971 PMCID: PMC11212587 DOI: 10.5500/wjt.v14.i2.92376] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/23/2024] [Revised: 04/12/2024] [Accepted: 04/26/2024] [Indexed: 06/13/2024] Open
Abstract
BACKGROUND Few studies have evaluated the frequency of and the reasons behind the refusal of listing liver transplantation candidates. AIM To assess the ineligibility rate for liver transplantation and its motivations. METHODS A single-center retrospective study was conducted on adult patients which entailed a formal multidisciplinary assessment for liver transplantation eligibility. The predictors for listing were evaluated using multivariable logistic regression. RESULTS In our center, 314 patients underwent multidisciplinary work-up before liver transplantation enlisting over a three-year period. The most frequent reasons for transplant evaluation were decompensated cirrhosis (51.6%) and hepatocellular carcinoma (35.7%). The non-listing rate was 53.8% and the transplant rate was 34.4% for the whole cohort. Two hundred and five motivations for ineligibility were collected. The most common contraindications were psychological (9.3%), cardiovascular (6.8%), and surgical (5.9%). Inappropriate or premature referral accounted for 76 (37.1%) cases. On multivariable analysis, a referral from another hospital (OR: 2.113; 95%CI: 1.259-3.548) served as an independent predictor of non-listing. CONCLUSION A non-listing decision occurred in half of our cohort and was based on an inappropriate or premature referral in one case out of three. The referral from another hospital was taken as a strong predictor of non-listing.
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Affiliation(s)
- Marco Biolato
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Luca Miele
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Giuseppe Marrone
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Claudia Tarli
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Antonio Liguori
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Rosaria Calia
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Giovanni Addolorato
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Salvatore Agnes
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Antonio Gasbarrini
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Maurizio Pompili
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
| | - Antonio Grieco
- Department of Medical and Surgical Sciences, CEMAD, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome 00168, Italy
- Department of Translational Medicine and Surgery, Catholic University of Sacred Heart, Rome 00168, Italy
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Rojas S, Rey Chaves CE, Robledo S, Conde D, Sabogal Olarte JC. Primary hepatic angiosarcoma: a systematic review. Ann Med Surg (Lond) 2024; 86:1601-1605. [PMID: 38463049 PMCID: PMC10923304 DOI: 10.1097/ms9.0000000000001584] [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: 10/26/2023] [Accepted: 11/24/2023] [Indexed: 03/12/2024] Open
Abstract
Background Hepatic angiosarcoma is a rare type of tumour. In adults, the diagnosis remains challenging as the clinical manifestations are generally nonspecific and are usually made too late when complications or metastases are already present, associated with a poor prognosis. Due to the lack of agreement regarding the optimal treatment approach, a comprehensive analysis of the evidence in the literature on the surgical and survival outcomes in terms of disease-free survival time (DFS) and overall survival (OS) for patients treated for primary hepatic angiosarcoma is needed. Study design A systematic review of articles published in Pubmed, EMBASE, and Scopus, from 2000 to 2023 with the search terms hepatic angiosarcoma and liver resection or hepatectomy. Pooled individual data concerning the prognosis following various therapies was analyzed. Results A total of 15 articles involving 886 patients were eligible for inclusion. The male population represents 66.2% (n=586) of the population, with a median age of 57 years (41-66). The median survival rate was 7 months. The median OS after surgical resection was 17.18 months (SD 12.6) vs. 3.72 months (SD 4.6) for patients treated without a surgical approach. The median DFS was 15.2 months (SD 11). Conclusion Primary liver angiosarcoma remains a surgical challenge with a poor prognosis even with complete surgical resection and adjuvant therapy. Surgical management shows increased overall survival in comparison with non-surgical management. Early diagnosis could change the natural history of the disease. The literature available is scarce, and further studies are required to create standardized diagnostic and treatment protocols.
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Affiliation(s)
| | | | - Sofía Robledo
- Estudiante de pregrado, Pontificia Universidad Javeriana, Facultad de Medicina
| | - Danny Conde
- Cirugia Hepatobiliar y pancreática, Hospital Universitario Mayor Méderi, Universidad el Rosario
| | - Juan Carlos Sabogal Olarte
- Cirugía Hepatobiliar y páncreatica, Pontificia Universidad Javeriana, Facultad de Medicina, Hospital Universitario San Ignacio, Bogotá, Colombia
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Fontinha GN, Gama JM, Caetano Oliveira R, Cipriano A. Embryonal Sarcoma of the Liver in the Adult: Challenges in the Diagnosis of a Rare Entity. GE PORTUGUESE JOURNAL OF GASTROENTEROLOGY 2024; 31:54-59. [PMID: 38476302 PMCID: PMC10928870 DOI: 10.1159/000528121] [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: 06/17/2022] [Accepted: 10/17/2022] [Indexed: 03/14/2024]
Abstract
Introduction Embryonal sarcoma of the liver (ESL) is a rare neoplasm of the liver occurring mainly in paediatric ages. Making the correct diagnosis can be challenging as the laboratory and radiological findings that are often nonspecific, and the tumour immunophenotype is poorly defined and even somewhat variable. Case Presentation A large epigastric mass was detected in a computerized tomography scan of a 43-year-old woman presenting with abdominal pain and bloating. The mass was biopsied and submitted to histopathological study. Microscopically the tumour had sarcomatoid features and showed multinucleated cells with periodic acid-Schiff (PAS)-positive globules. Immunostaining revealed positivity for vimentin, CD10, glypican-3, and α1-antitrypsin and negativity for keratins, muscle, adipocytic, and melanocytic differentiation markers. The patient was then submitted to a left hepatectomy with similar histological findings. Discussion ESL in adults is a rarity and its diagnosis requires the exclusion of other entities. While some microscopic features are very common, they remain nonspecific. The main feature is the presence of multinucleated cells with PAS-positive hyaline globules. While ancillary testing is key, the immunophenotype also lacks specificity and ESL may have variable staining for glypican-3 and epithelial or muscle differentiation markers. Although it has been described for more than 3 decades, the prognosis and optimal treatment are still not well defined, but surgery has yielded favourable results.
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6
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Krendl FJ, Bellotti R, Sapisochin G, Schaefer B, Tilg H, Scheidl S, Margreiter C, Schneeberger S, Oberhuber R, Maglione M. Transplant oncology - Current indications and strategies to advance the field. JHEP Rep 2024; 6:100965. [PMID: 38304238 PMCID: PMC10832300 DOI: 10.1016/j.jhepr.2023.100965] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/27/2023] [Revised: 10/31/2023] [Accepted: 11/04/2023] [Indexed: 02/03/2024] Open
Abstract
Liver transplantation (LT) was originally described by Starzl as a promising strategy to treat primary malignancies of the liver. Confronted with high recurrence rates, indications drifted towards non-oncologic liver diseases with LT finally evolving from a high-risk surgery to an almost routine surgical procedure. Continuously improving outcomes following LT and evolving oncological treatment strategies have driven renewed interest in transplant oncology. This is not only reflected by constant refinements to the criteria for LT in patients with HCC, but especially by efforts to expand indications to other primary and secondary liver malignancies. With new patient-centred oncological treatments on the rise and new technologies to expand the donor pool, the field has the chance to come full circle. In this review, we focus on the concept of transplant oncology, current indications, as well as technical and ethical aspects in the context of donor organs as precious resources.
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Affiliation(s)
- Felix J. Krendl
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Ruben Bellotti
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Gonzalo Sapisochin
- Multi-Organ Transplant Program, University Health Network, Toronto, Ontario, Canada
| | - Benedikt Schaefer
- Department of Medicine I, Gastroenterology, Hepatology and Endocrinology, Medical University of Innsbruck, Austria
| | - Herbert Tilg
- Department of Medicine I, Gastroenterology, Hepatology and Endocrinology, Medical University of Innsbruck, Austria
| | - Stefan Scheidl
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Christian Margreiter
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Stefan Schneeberger
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Rupert Oberhuber
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
| | - Manuel Maglione
- Department of Visceral, Transplant and Thoracic Surgery, Center for Operative Medicine, Medical University of Innsbruck, Austria
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Finney N, Tran T, Hasjim BJ, Jakowatz J, Chiao E, Eng O, Wolf R, Jutric Z, Yamamoto M, Tran TB. Primary sarcoma of the liver: A nationwide analysis of a rare mesenchymal tumor. J Surg Oncol 2024; 129:358-364. [PMID: 37796036 DOI: 10.1002/jso.27477] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2023] [Revised: 09/10/2023] [Accepted: 09/23/2023] [Indexed: 10/06/2023]
Abstract
BACKGROUND AND OBJECTIVES Primary liver sarcomas are rare malignancies. Prognostic factors associated with long-term survival remain poorly understood. The objective of this study is to determine factors associated with long-term survival. METHODS The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with visceral sarcoma arising from the liver. Demographic factors, tumor characteristics, resection status, and survival were evaluated. Multivariate Cox regression analysis was performed to determine predictors of survival. RESULTS A total of 743 patients with primary hepatic sarcoma were identified. The median tumor size was 10 cm. Only 30% (n = 221) of patients in the cohort underwent surgery. The 5-year overall survival rates were 47.9% for localized disease, 29.5% for regional disease, and 16.5% for distant disease, p < 0.001. Among patients who underwent surgical resection, patients with embryonal sarcoma had better 5-year survival compared with angiosarcoma and other histologic subtypes. On multivariate analysis, surgery was associated with improved survival, while older age, higher stage, and angiosarcoma histology were the strongest independent predictors of poor survival. CONCLUSIONS Surgery remains the mainstay of treatment for this rare malignancy but is performed in less than one-third of patients. Angiosarcoma histology is associated with worse overall survival, while surgical resection remains the strongest predictor of improved overall survival.
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Affiliation(s)
- Nicole Finney
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Tu Tran
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Bima J Hasjim
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - James Jakowatz
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Elaine Chiao
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Oliver Eng
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Ronald Wolf
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Zeljika Jutric
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Maki Yamamoto
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
| | - Thuy B Tran
- Department of Surgery, Division of Surgical Oncology, University of California Irvine, Orange, California, USA
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Dobrindt EM, Öllinger R, Flörcken A, Märdian S, Schmelzle M, Lurje G, Pratschke J, Schoening W. [Primary Hepatic Sarcoma - Recommendations for Therapy]. Zentralbl Chir 2023; 148:165-179. [PMID: 35211924 DOI: 10.1055/a-1728-6939] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Affiliation(s)
- Eva Maria Dobrindt
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Robert Öllinger
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Anne Flörcken
- Medizinische Klinik m.S. Hämatologie, Onkologie und Tumorimmunologie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Sven Märdian
- Zentrum für Muskuloskeletale Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Moritz Schmelzle
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Georg Lurje
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Johann Pratschke
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
| | - Wenzel Schoening
- Klinik für Chirurgie, Charité - Universitätsmedizin Berlin, Berlin, Deutschland
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9
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Dogeas E. Hepatic epithelioid hemangioendothelioma: pitfalls in the treatment of a rare liver malignancy. Transl Gastroenterol Hepatol 2023; 8:3. [PMID: 36704650 PMCID: PMC9813652 DOI: 10.21037/tgh-22-80] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/01/2022] [Accepted: 09/13/2022] [Indexed: 01/29/2023] Open
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Tansir G, Rastogi S, Barwad A, Yadav R, Shamim SA, Dhamija E, Pandey R, Garg R, Shrivastava S. Management and outcomes of advanced hemangioendothelioma at a medical oncology clinic in an Indian tertiary care center. Future Sci OA 2022; 8:FSO827. [PMID: 36874373 PMCID: PMC9979162 DOI: 10.2144/fsoa-2021-0132] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/14/2022] [Accepted: 01/10/2023] [Indexed: 02/05/2023] Open
Abstract
AIM Hemangioendotheliomas (HEs) are malignant vascular tumors with sparse descriptions in literature owing to their rarity. STUDY DESIGN Ours is a retrospective study among patients of advanced HEs registered between September 2015 and April 2021. RESULTS There were 13 patients with median age 34.6 (range: 4-69 years), male preponderance (69%) and predominant subtype of epithelioid HE (76.9%). Common primary sites were viscera (46.2%) and bone (30.8%). Tyrosine kinase inhibitors (TKIs) yielded objective responses in 30% patients whereas chemotherapy only produced disease stabilization in 7.7%. CONCLUSION We recognize an aggressive subset of HEs with manifestations such as acute liver failure and splenic rupture. Currently no biomarkers predict the efficacy of TKIs over chemotherapy; however, TKIs showed promising outcomes in this series.
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Affiliation(s)
- Ghazal Tansir
- Department of Medical Oncology, BRA IRCH, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Sameer Rastogi
- Sarcoma Medical Oncology Clinic, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Adarsh Barwad
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Rajni Yadav
- Department of Pathology, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Shamim Ahmed Shamim
- Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Ekta Dhamija
- Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Rambha Pandey
- Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Rakesh Garg
- Department of Oncoanesthesia & Palliative Medicine, All India Institute of Medical Sciences, New Delhi, 110029, India
| | - Shakti Shrivastava
- Sarcoma Medical Oncology Clinic, All India Institute of Medical Sciences, New Delhi, 110029, India
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Yang S, Liao Z, Li T, Liu H, Ren Z, Wu H, Zhao J, Teng S, Xing R, Yang Y, Yang J. Clinicopathologic features, prognostic factors, and outcomes of visceral sarcomas: A retrospective 12-year single-center study. Front Oncol 2022; 12:1029913. [DOI: 10.3389/fonc.2022.1029913] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/28/2022] [Accepted: 10/17/2022] [Indexed: 11/09/2022] Open
Abstract
BackgroundVisceral sarcomas are a rare form of soft tissue sarcoma. This study aimed to evaluate the survival and prognostic factors and effective treatments for visceral sarcomas.MethodsAll patients with visceral sarcoma referred to our center between January 2010 and December 2021 were retrospectively analyzed. The Kaplan-Meier method and a log-rank test were used for survival analysis.ResultsA total of 53 patients with visceral sarcoma were analyzed in this study with the median age at diagnosis of 57 (range, 24-77) years. Among them, 37 (69.8%) and 16 (30.2%) patients had localized and metastatic diseases at the initial presentation, respectively, and 44 patients underwent surgical resection. The median follow-up, event-free survival (EFS) and overall survival (OS) were 63.0 (range, 2-130), 42.0 months (95% confidence interval [CI] 10.879-73.121) and 45.0 months (95% CI 9.938-80.062), respectively. The 5-year EFS and OS rates were 44% and 46%, respectively. Univariate analysis of prognostic indicators illustrated that metastasis at presentation, surgery, surgical margin and the types of surgery were significantly associated with OS and EFS. In this study, combined chemotherapy or radiotherapy had no effects on EFS and OS.ConclusionPrimary visceral sarcoma is an uncommon and aggressive malignant tumor with a higher rate of local recurrence. In the largest cohort of visceral sarcomas in China to date, we identified metastases at presentation, surgery, surgical margin, and the types of surgery as independent predictors of survival. The combination of chemotherapy and radiotherapy did not affect survival.
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Establishment and Validation of a Nomogram Prognostic Model for Epithelioid Hemangioendothelioma. JOURNAL OF ONCOLOGY 2022; 2022:6254563. [PMID: 36245980 PMCID: PMC9560816 DOI: 10.1155/2022/6254563] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 05/24/2022] [Revised: 08/14/2022] [Accepted: 08/20/2022] [Indexed: 11/17/2022]
Abstract
Background. Epithelioid hemangioendothelioma (EHE) is an ultrarare vascular sarcoma. At present, the epidemiological and clinical characteristics and prognostic factors are still unclear. Our study attempted to describe clinical features, investigate the prognostic indicators, and establish the nomogram prediction model based on the Surveillance, Epidemiology, and End Results (SEER) database for EHE patients. Methods. The patients diagnosed with EHE from 1986 to 2018 were collected from the SEER database and were randomly divided into a training group and a validation group at a ratio of 7 : 3. The Cox proportional hazard models were used to determine the independent factors affecting prognosis and establish a nomogram prognostic model to predict the survival rates for patients with EHE. The accuracy and discriminative ability of the model were measured using the concordance index, receiver operating characteristic curves, and calibration curves. The clinical applicability and application value of the model were evaluated by decision curve analysis. Results. The overall age-adjusted incidence of EHE was 0.31 patients per 1,000,000 individuals, with a statistically significant difference per year. Overall survival at 1, 5, and 10 years for all patients was 76.5%, 57.4%, and 48.2%, respectively. Multivariate Cox regression analysis identified age, tumour stage, degree of tissue differentiation, surgical treatment, chemotherapy, and radiotherapy as independent factors affecting prognosis (
). The C-index values for our nomogram model of training group and validation group were 0.752 and 0.753, respectively. The calibration curve was in good agreement with the actual observation results, suggesting that the prediction model has good accuracy. The decision curve analysis indicated a relatively large net benefit. Conclusions. The nomogram model may play an important role in predicting the survival rate for EHE patients, with good concordance and accuracy, and can be applied in clinical practice.
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Zhou L, Li SY, Chen C, Xu J. Hemorrhagic shock after contrast-enhanced ultrasound-guided liver biopsy: A case report of primary hepatic angiosarcoma. JOURNAL OF CLINICAL ULTRASOUND : JCU 2022; 50:698-702. [PMID: 34964135 DOI: 10.1002/jcu.23114] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 07/27/2021] [Revised: 11/15/2021] [Accepted: 12/03/2021] [Indexed: 06/14/2023]
Abstract
Primary hepatic angiosarcoma (PHA) is a rare, highly aggressive malignancy. There is no specificity in clinical manifestations, laboratory examinations and imaging examinations. The definitive diagnosis of PHA depends on pathological analysis. The gold standard method to obtain specimens is percutaneous transhepatic biopsy under the guidance of contrast-enhanced ultrasound (CEUS). However, CEUS-guided biopsy for PHA is controversial. If a biopsy is necessary, a comprehensive preoperative evaluation is essential. In addition, CEUS has an auxiliary value in diagnosing PHA. In this case, we present an elderly woman who underwent CEUS-guided liver mass biopsy. The patient developed hemorrhagic shock after biopsy.
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Affiliation(s)
- Ling Zhou
- Department of Ultrasound, Sir Run Run Shaw Hospital, Zhejiang University, School of Medicine, Hangzhou, China
| | - Shi-Yan Li
- Department of Ultrasound, Sir Run Run Shaw Hospital, Zhejiang University, School of Medicine, Hangzhou, China
| | - Chao Chen
- Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University, School of Medicine, Hangzhou, China
| | - Jing Xu
- Department of Pathology, Sir Run Run Shaw Hospital, Zhejiang University, School of Medicine, Hangzhou, China
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Ilmer M, Guba MO. Liver Transplant Oncology: Towards Dynamic Tumor-Biology-Oriented Patient Selection. Cancers (Basel) 2022; 14:cancers14112662. [PMID: 35681642 PMCID: PMC9179475 DOI: 10.3390/cancers14112662] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2022] [Revised: 05/10/2022] [Accepted: 05/19/2022] [Indexed: 02/04/2023] Open
Abstract
While liver transplantation was initially considered as a curative treatment modality only for hepatocellular carcinoma, the indication has been increasingly extended to other tumor entities over recent years, most recently to the treatment of non-resectable colorectal liver metastases. Although oncologic outcomes after liver transplantation (LT) are consistently good, organ shortage forces stringent selection of suitable candidates. Dynamic criteria based on tumor biology fulfill the prerequisite of an individual oncological prediction better than traditional morphometric criteria based on tumor burden. The availability of specific (neo-)adjuvant therapies and customized modern immunosuppression may further contribute to favorable post-transplantation outcomes on the one hand and simultaneously open the path to LT as a curative option for advanced stages of tumor patients. Herein, we provide an overview of the oncological LT indications, the selection process, and expected oncological outcome after LT.
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Affiliation(s)
- Matthias Ilmer
- Department of General, Visceral and Transplantation Surgery, Hospital of the University of Munich, Ludwig-Maximilians-University (LMU), 81377 Munich, Germany;
- German Cancer Consortium (DKTK), Partner Site Munich, German Cancer Research Center (DKFZ), 69120 Heidelberg, Germany
- Transplantation Center Munich, Ludwig-Maximilians-University Munich, Campus Grosshadern, 81377 Munich, Germany
- Liver Center Munich, Ludwig-Maximilians-University Munich, 81377 Munich, Germany
- Correspondence:
| | - Markus Otto Guba
- Department of General, Visceral and Transplantation Surgery, Hospital of the University of Munich, Ludwig-Maximilians-University (LMU), 81377 Munich, Germany;
- Transplantation Center Munich, Ludwig-Maximilians-University Munich, Campus Grosshadern, 81377 Munich, Germany
- Liver Center Munich, Ludwig-Maximilians-University Munich, 81377 Munich, Germany
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15
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Treatment Outcomes for Primary Hepatic Angiosarcoma: National Cancer Database Analysis 2004-2014. Curr Oncol 2022; 29:3637-3646. [PMID: 35621682 PMCID: PMC9139369 DOI: 10.3390/curroncol29050292] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/24/2022] [Revised: 05/13/2022] [Accepted: 05/16/2022] [Indexed: 11/16/2022] Open
Abstract
Background: To determine the risk of mortality and factors associated with survival amongst patients diagnosed with primary hepatic angiosarcoma (PHA). Methods: All patients diagnosed with hepatocellular carcinoma (HCC) or PHA from 2004 to 2014 were identified from the National Cancer Database (NCDB). Further analysis was performed within the cohort of patients with PHA to assess the impact of surgery, chemotherapy, radiation, and facility type on overall survival (OS). A multivariable analysis using the Cox proportional methods and a survival analysis using the Kaplan−Meier method were used. Results: A total of 117,633 patients with HCC were identified, out of whom 346 patients had PHA. Patients with PHA had a mean age of 62.9 years (SD 13.7), the majority were men (64.7%), white (85.8%), and had a Charlson comorbidity index (CCI) of zero (66.2%). A third of the patients with PHA (35.7%) received chemotherapy, and 14.6% underwent a surgical resection. The median survival was 1.9 months (1.8−2.4 months) compared to patients with HCC (10.4 months, 10.2−10.5) (aHR-2.41, 95% CI: 2.10−2.77, p < 0.0001). Surgical resection was associated with a higher median survival (7.7 versus 1.8 months, aHR-0.23, 95% CI: 0.15−0.37, p < 0.0001). A receipt of chemotherapy was associated with a higher median survival than no chemotherapy (5.1 versus 1.2 months, aHR-0.44, 95% CI: 0.32−0.60, p < 0.0001), although the survival benefit did not persist long term. Conclusion: PHA is associated with poor outcomes. A surgical resection and chemotherapy are associated with improved survival outcomes; however, the long-term benefits of chemotherapy are limited.
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16
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Houben P, Schimmack S, Unterrainer C, Döhler B, Mehrabi A, Süsal C. Rare Malignant Indications for Liver Transplantation: A Collaborative Transplant Study Report. Front Surg 2021; 8:678392. [PMID: 34926560 PMCID: PMC8678034 DOI: 10.3389/fsurg.2021.678392] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/09/2021] [Accepted: 10/27/2021] [Indexed: 12/04/2022] Open
Abstract
Introduction: Hepatocellular carcinoma (HCC) is by far the leading malignant indication for liver transplantation (LT). Few other malignancies, including cholangiocellular carcinoma (CCC), metastases from neuroendocrine tumors (NET), and sarcomas of the liver (LSAR), also are commonly accepted indications for LT. However, there is limited information on their outcome after LT. Methods: Graft and patient survival in 14,623 LTs performed in patients with hepatocellular carcinoma, CCC, NET, and LSAR from 1988 to 2017 and reported to the Collaborative Transplant Study were analyzed. Results: The study group consisted of 13,862 patients who had HCC (94.8%), 498 (3.4%) who had CCC, 100 (0.7%) who had NET, and 163 (1.1%) who had LSAR. CCC patients showed a 5-year graft survival rate of 32.1%, strikingly lower than the 63.2% rate in HCC, 51.6% rate in NET, and 64.5% rate in LSAR patients (P < 0.001 for all vs. CCC). Multivariable Cox regression analysis revealed a significantly higher risk of graft loss and death due to cancer during the first five post-transplant years in CCC vs. HCC patients (HR 1.77 and 2.56; P < 0.001 for both). The same risks were increased also in NET and LSAR patients but did not reach statistical significance. Conclusion: Among patients with rare malignant indications for LT, CCC patients showed significantly impaired graft as well as patient survival compared to HCC patients. The observed differences might challenge traditional decision-making processes for LT indication and palliative treatment in specific hepatic malignancies.
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Affiliation(s)
- Philipp Houben
- Department of General, Visceral, and Transplant Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Simon Schimmack
- Department of General, Visceral, and Transplant Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | | | - Bernd Döhler
- Institute of Immunology, Heidelberg University Hospital, Heidelberg, Germany
| | - Arianeb Mehrabi
- Department of General, Visceral, and Transplant Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Caner Süsal
- Institute of Immunology, Heidelberg University Hospital, Heidelberg, Germany.,Transplant Immunology Research Center of Excellence, Koç Üniversitesi, Istanbul, Turkey
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17
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Martínez C, Lai JK, Ramai D, Facciorusso A, Gao Z. Cancer registry study of malignant hepatic vascular tumors: hepatic angiosarcomas and hepatic epithelioid hemangioendotheliomas. Cancer Med 2021; 10:8883-8890. [PMID: 34850580 PMCID: PMC8683533 DOI: 10.1002/cam4.4403] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/29/2021] [Revised: 09/14/2021] [Accepted: 10/01/2021] [Indexed: 12/21/2022] Open
Abstract
BACKGROUND Malignant vascular tumors (MVTs) are rare and often misdiagnosed due to wide range of clinical presentations, varied histology, and exquisite imagining features. We aim to characterize two different types of MVTs of the liver: hepatic angiosarcomas (HA) and hepatic epithelioid hemangioendotheliomas (HEHE). METHODS Data on HA and HEHE between 1975 and 2016 were extracted from the SEER database and analyzed. RESULTS A total of 366 patients with HA were identified where 64.2% were male and 79% of White race. The median age at diagnosis was 64 ± 16.2 years. Distant metastasis was found in 24% of patients, regional disease in 22.1%, and localized disease in 21.3%. The median overall survival for HA was 2 months. For HEHE, 120 cases were identified, 32.5% were male and 80% of White race. The median age of diagnosis was 51 ± 16.8 years. Distant metastasis was found in 37.5% of patients, regional disease in 27.5%, and localized disease in 20%. The median overall survival was 182 months. CONCLUSION Patients' demographics such as race, age, and gender may assist in elucidating distinct subtypes of MVTs. HA is an aggressive tumor despite intervention. Patients with HEHE tumors have significantly better survival compared to patients with HA. Further studies are needed to deepen our knowledge about the histopathology of these tumors, the outcomes of liver transplantation as a therapeutic alternative, and available molecular targets for MVTs.
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Affiliation(s)
- Constanza Martínez
- Department of Radiation OncologyMcGill UniversityMontrealQuebecCanada
- Department of PathologyMcGill UniversityMontrealQuebecCanada
| | - Jonathan K. Lai
- Department of PathologyMcGill UniversityMontrealQuebecCanada
| | - Daryl Ramai
- Department of MedicineThe Brooklyn Hospital CenterBrooklynNew YorkUSA
- Division of Gastroenterology & HepatologyCHI Health Creighton University Medical CenterOmahaNebraskaUSA
| | - Antonio Facciorusso
- Division of GastroenterologyUniversity of Nebraska Medical CenterOmahaNebraskaUSA
- Division of GastroenterologyMoffitt Cancer CenterUniversity of South FloridaTampaFloridaUSA
- Section of GastroenterologyDepartment of Medical SciencesUniversity of FoggiaFoggiaItaly
| | - Zu‐Hua Gao
- Department of PathologyMcGill UniversityMontrealQuebecCanada
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18
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Lang SA, Bednarsch J, Czigany Z, Joechle K, Kroh A, Amygdalos I, Strnad P, Bruns T, Heise D, Ulmer F, Neumann UP. Liver transplantation in malignant disease. World J Clin Oncol 2021; 12:623-645. [PMID: 34513597 PMCID: PMC8394155 DOI: 10.5306/wjco.v12.i8.623] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Revised: 06/15/2021] [Accepted: 07/23/2021] [Indexed: 02/06/2023] Open
Abstract
Liver transplantation for malignant disease has gained increasing attention as part of transplant oncology. Following the implementation of the Milan criteria, hepatocellular carcinoma (HCC) was the first generally accepted indication for transplantation in patients with cancer. Subsequently, more liberal criteria for HCC have been developed, and research on this topic is still ongoing. The evident success of liver transplantation for HCC has led to the attempt to extend its indication to other malignancies. Regarding perihilar cholangiocarcinoma, more and more evidence supports the use of liver transplantation, especially after neoadjuvant therapy. In addition, some data also show a benefit for selected patients with very early stage intrahepatic cholangiocarcinoma. Hepatic epithelioid hemangioendothelioma is a very rare but nonetheless established indication for liver transplantation in primary liver cancer. In contrast, patients with hepatic angiosarcoma are currently not considered to be optimal candidates. In secondary liver tumors, neuroendocrine cancer liver metastases are an accepted but comparability rare indication for liver transplantation. Recently, some evidence has been published supporting the use of liver transplantation even for colorectal liver metastases. This review summarizes the current evidence for liver transplantation for primary and secondary liver cancer.
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Affiliation(s)
- Sven Arke Lang
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Jan Bednarsch
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Zoltan Czigany
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Katharina Joechle
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Andreas Kroh
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Iakovos Amygdalos
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Pavel Strnad
- Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Tony Bruns
- Department of Internal Medicine III, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Daniel Heise
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Florian Ulmer
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
| | - Ulf Peter Neumann
- Department of Surgery and Transplantation, University Hospital RWTH Aachen, Aachen 52074, Germany
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19
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汪 辉, 李 俏, 林 乐, 李 洁, 周 晓, 何 建. 35例上皮样血管内皮瘤的临床表现及预后影响因素分析. SICHUAN DA XUE XUE BAO. YI XUE BAN = JOURNAL OF SICHUAN UNIVERSITY. MEDICAL SCIENCE EDITION 2021; 52:716-720. [PMID: 37876528 PMCID: PMC10409403 DOI: 10.12182/20210760204] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 09/15/2020] [Indexed: 10/26/2023]
Abstract
目的 分析总结一种罕见的低度恶性肿瘤——上皮样血管内皮瘤（EHE）的临床表现、总体生存率及相关预后因素。 方法 本研究纳入了2009年6月–2020年3月在四川大学华西医院院病理诊断为EHE的35例患者，分析其临床表现。采用Kaplan-Meier生存曲线和COX回归分析比较各亚组间生存率以及影响EHE预后的危险因素。 结果 EHE平均发病年龄为41岁（3~70岁），男女发病率比为1∶1.5（14/21）。可多发于各个器官，最常见的受累器官是肝脏（63%）。在增强CT和MRI影像学上，EHE的特征包括“靶征”和“棒棒糖征”，靠近肝周生长的肿瘤常存在肝包膜回缩征。组织病理学检查HE染色可见大小不一，形态多样的血管腔结构，腔内散在分布红细胞，肿瘤细胞呈浸润性生长，呈条索状，巢状分布；免疫组化染色提示CD31、CD34、8因子、ERG阳性，荧光免疫杂交（FISH）检查提示，钙调蛋白转录激活因子1（CAMTA 1）阳性率为66.6%（8/12），转录因子E3（TFE 3）阳性率为18.2%（2/11）。患者1年、3年、5年生存率分别为80.00%、68.57%、62.86%。COX回归分析发现Ki-67是影响预后的独立因素（ P =0.034，相对危险度为5.809，95%置信区间1.14～29.55）。 结论 EHE恶性程度较低，Ki-67≥10%的EHE患者预后较差。
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Affiliation(s)
- 辉 汪
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
| | - 俏琦 李
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
| | - 乐德 林
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
| | - 洁琅 李
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
| | - 晓娟 周
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
| | - 建萍 何
- 四川大学华西医院 腹部肿瘤科 （成都 610041）
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20
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Martínez-Trufero J, Cruz Jurado J, Gómez-Mateo MC, Bernabeu D, Floría LJ, Lavernia J, Sebio A, García Del Muro X, Álvarez R, Correa R, Hernández-León CN, Marquina G, Hindi N, Redondo A, Martínez V, Asencio JM, Mata C, Valverde Morales CM, Martin-Broto J. Uncommon and peculiar soft tissue sarcomas: Multidisciplinary review and practical recommendations for diagnosis and treatment. Spanish group for Sarcoma research (GEIS - GROUP). Part I. Cancer Treat Rev 2021; 99:102259. [PMID: 34311246 DOI: 10.1016/j.ctrv.2021.102259] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/18/2021] [Revised: 07/03/2021] [Accepted: 07/06/2021] [Indexed: 12/22/2022]
Affiliation(s)
| | - Josefina Cruz Jurado
- Hospital Universitario Canarias, Medical Oncology Department, Santa Cruz de Tenerife, Spain
| | | | - Daniel Bernabeu
- Hospital Universitario La Paz, Radiology Department, Madrid, Spain
| | - Luis Javier Floría
- Hospital Universitario Miguel Servet, Orthopedic and Traumatology Department, Zaragoza, Spain
| | - Javier Lavernia
- Instituto Valenciano de Oncología, Medical Oncology Department, Valencia, Spain
| | - Ana Sebio
- Hospital Universitario Santa Creu i Sant Pau, Medical Oncology Department, Barcelona, Spain
| | | | - Rosa Álvarez
- Hospital Universitario Gregorio Marañón, Medical Oncology Department, Madrid, Spain
| | - Raquel Correa
- Hospital Virgen de la Victoria, Radiation Oncology Department, Malaga, Spain
| | | | - Gloria Marquina
- Hospital Universitario Clínico San Carlos, Medical Oncology Department, Madrid, Spain
| | - Nadia Hindi
- University Hospital "Fundacion Jimenez Diaz" Madrid, Medical Oncology Department, Madrid, Research Institute FJD-UAM, Madrid (Spain), TBsarc, CITIUS III, Seville, Spain
| | - Andrés Redondo
- Hospital Universitario La Paz, Medical Oncology Department, Madrid, Spain
| | - Virginia Martínez
- Hospital Universitario La Paz, Medical Oncology Department, Madrid, Spain
| | | | - Cristina Mata
- Hospital Universitario Gregorio Marañón, Pediatric and Adolescent Hemato-oncology Department, Madrid, Spain
| | | | - Javier Martin-Broto
- University Hospital "Fundacion Jimenez Diaz" Madrid, Medical Oncology Department, Madrid, Research Institute FJD-UAM, Madrid (Spain), TBsarc, CITIUS III, Seville, Spain
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21
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Stacchiotti S, Miah AB, Frezza AM, Messiou C, Morosi C, Caraceni A, Antonescu CR, Bajpai J, Baldini E, Bauer S, Biagini R, Bielack S, Blay JY, Bonvalot S, Boukovinas I, Bovee JVMG, Boye K, Brodowicz T, Callegaro D, De Alava E, Deoras-Sutliff M, Dufresne A, Eriksson M, Errani C, Fedenko A, Ferraresi V, Ferrari A, Fletcher CDM, Garcia Del Muro X, Gelderblom H, Gladdy RA, Gouin F, Grignani G, Gutkovich J, Haas R, Hindi N, Hohenberger P, Huang P, Joensuu H, Jones RL, Jungels C, Kasper B, Kawai A, Le Cesne A, Le Grange F, Leithner A, Leonard H, Lopez Pousa A, Martin Broto J, Merimsky O, Merriam P, Miceli R, Mir O, Molinari M, Montemurro M, Oldani G, Palmerini E, Pantaleo MA, Patel S, Piperno-Neumann S, Raut CP, Ravi V, Razak ARA, Reichardt P, Rubin BP, Rutkowski P, Safwat AA, Sangalli C, Sapisochin G, Sbaraglia M, Scheipl S, Schöffski P, Strauss D, Strauss SJ, Sundby Hall K, Tap WD, Trama A, Tweddle A, van der Graaf WTA, Van De Sande MAJ, Van Houdt W, van Oortmerssen G, Wagner AJ, Wartenberg M, Wood J, Zaffaroni N, Zimmermann C, Casali PG, Dei Tos AP, Gronchi A. Epithelioid hemangioendothelioma, an ultra-rare cancer: a consensus paper from the community of experts. ESMO Open 2021; 6:100170. [PMID: 34090171 PMCID: PMC8182432 DOI: 10.1016/j.esmoop.2021.100170] [Citation(s) in RCA: 106] [Impact Index Per Article: 26.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2021] [Revised: 05/07/2021] [Accepted: 05/11/2021] [Indexed: 12/22/2022] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is an ultra-rare, translocated, vascular sarcoma. EHE clinical behavior is variable, ranging from that of a low-grade malignancy to that of a high-grade sarcoma and it is marked by a high propensity for systemic involvement. No active systemic agents are currently approved specifically for EHE, which is typically refractory to the antitumor drugs used in sarcomas. The degree of uncertainty in selecting the most appropriate therapy for EHE patients and the lack of guidelines on the clinical management of the disease make the adoption of new treatments inconsistent across the world, resulting in suboptimal outcomes for many EHE patients. To address the shortcoming, a global consensus meeting was organized in December 2020 under the umbrella of the European Society for Medical Oncology (ESMO) involving >80 experts from several disciplines from Europe, North America and Asia, together with a patient representative from the EHE Group, a global, disease-specific patient advocacy group, and Sarcoma Patient EuroNet (SPAEN). The meeting was aimed at defining, by consensus, evidence-based best practices for the optimal approach to primary and metastatic EHE. The consensus achieved during that meeting is the subject of the present publication.
This consensus paper provides key recommendations on the management of epithelioid hemangioendothelioma (EHE). Recommendations followed a consensus meeting between experts and a representative of the EHE advocacy group and SPAEN. Authorship includes a multidisciplinary group of experts from different institutions from Europe, North America and Asia.
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Affiliation(s)
- S Stacchiotti
- Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy.
| | - A B Miah
- The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
| | - A M Frezza
- Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - C Messiou
- Department of Radiology, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
| | - C Morosi
- Radiology Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - A Caraceni
- Palliative Care Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - C R Antonescu
- Department of Pathology, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, USA
| | - J Bajpai
- Medical Oncology Department, Tata Memorial Centre, Homi Bhabha National Institute, Mumbai, India
| | - E Baldini
- Department of Radiation Oncology, Dana-Farber Cancer Center/Brigham and Women's Hospital, Boston, USA
| | - S Bauer
- Department of Medical Oncology, West German Cancer Center, Sarcoma Center, University of Duisburg-Essen, University Hospital, Essen, Germany
| | - R Biagini
- Orthopaedic Department, Regina Elena National Cancer Institute, Rome, Italy
| | - S Bielack
- Klinikum Stuttgart - Olgahospital, Zentrum für Kinder-, Jugend- und Frauenmedizin, Stuttgart Cancer Center, Pädiatrische Onkologie, Hämatologie, Immunologie, Stuttgart, Germany
| | - J Y Blay
- Department of Medical Oncology, Centre Leon Berard, Université Claude Bernard Lyon, Unicancer, Lyon, France
| | - S Bonvalot
- Department of Surgical Oncology, Institut Curie, Université Paris Sciences et Lettres, Paris, France
| | | | - J V M G Bovee
- Department of Pathology, Leiden University Medical Center, Leiden, The Netherlands
| | - K Boye
- Department of Oncology, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway
| | - T Brodowicz
- Medical University Vienna & General Hospital Department of Internal Medicine 1/Oncology, Vienna, Austria
| | - D Callegaro
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - E De Alava
- Institute of Biomedicine of Sevilla (IBiS), Virgen del Rocio University Hospital/CSIC/University of Sevilla/CIBERONC, Seville, Spain; Department of Normal and Pathological Cytology and Histology, School of Medicine, University of Seville, Seville, Spain
| | | | - A Dufresne
- Department of Medical Oncology, Centre Leon Berard, Université Claude Bernard Lyon, Unicancer, Lyon, France
| | - M Eriksson
- Department of Oncology, Skane University Hospital and Lund University, Lund, Sweden
| | - C Errani
- Orthopaedic Service, Musculoskeletal Oncology Department, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
| | - A Fedenko
- Medical Oncology Division, P.A. Herzen Cancer Research Institute, Moscow, Russian Federation
| | - V Ferraresi
- Sarcomas and Rare Tumors Unit, IRCCS Regina Elena National Cancer Institute, Rome, Italy
| | - A Ferrari
- Paediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - C D M Fletcher
- Department of Pathology Brigham & Women's Hospital, Boston, USA
| | - X Garcia Del Muro
- University of Barcelona and Genitourinary Cancer and Sarcoma Unit Institut Català d'Oncologia, Hospitalet, Barcelona, Spain
| | - H Gelderblom
- Department of Medical Oncology, Leiden University Medical Center, Leiden, The Netherlands
| | - R A Gladdy
- University of Toronto and Lunenfeld-Tanenbaum Research Institute, Toronto, Canada
| | - F Gouin
- Department of Surgery, Centre Leon Berard, Lyon, France
| | - G Grignani
- Division of Medical Oncology, Candiolo Cancer Institute, FPO - IRCCS, Candiolo, Italy
| | - J Gutkovich
- The EHE Foundation, Wisconsin, USA; NUY Langone Medical Center, New York, USA
| | - R Haas
- Department of Radiotherapy, the Netherlands Cancer Institute, Amsterdam, the Netherlands; Department of Radiotherapy, the Leiden University Medical Center, Leiden, the Netherlands
| | - N Hindi
- Group of Advanced Therapies and Biomarkers in Sarcoma, Institute of Biomedicine of Seville (IBIS, HUVR, CSIC, Universidad de Sevilla), Seville, Spain
| | - P Hohenberger
- Division of Surgical Oncology & Thoracic Surgery, Mannheim University Medical Center, University of Heidelberg, Heidelberg, Germany
| | - P Huang
- Division of Molecular Pathology, The Institute of Cancer Research, London, UK
| | - H Joensuu
- Department of Oncology, Helsinki University Hospital & Helsinki University, Helsinki, Finland
| | - R L Jones
- Department of Cancer, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
| | - C Jungels
- Institut Jules Bordet, Université Libre de Bruxelles, Brussels, Belgium
| | - B Kasper
- University of Heidelberg, Mannheim University Medical Center, Sarcoma Unit, Mannheim, Germany
| | - A Kawai
- Musculoskeletal Oncology and Rehabilitation Medicine, Rare Cancer Center National Cancer Center Hospital, Tokyo, Japan
| | - A Le Cesne
- International Department, Gustave Roussy, Villejuif, France
| | - F Le Grange
- UCLH - University College London Hospitals NHS Foundation Trust, London, UK
| | - A Leithner
- Department of Orthopaedics and Trauma Medical University Graz, Graz, Austria
| | - H Leonard
- Chair of Trustees of the EHE Rare Cancer Charity (UK), Charity number 1162472
| | - A Lopez Pousa
- Medical Oncology Department, Hospital Universitario Santa Creu i Sant Pau, Barcelona, Spain
| | - J Martin Broto
- Hospital Universitario Fundación Jimenez Diaz, Madrid, Spain
| | - O Merimsky
- Unit of Soft Tissue and Bone Oncology, Division of Oncology, Tel-Aviv Medical Center affiliated with Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - P Merriam
- Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, USA
| | - R Miceli
- Department of Clinical Epidemiology and Trial Organisation, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
| | - O Mir
- Sarcoma Group, Gustave Roussy, Villejuif, France
| | - M Molinari
- University of Pittsburgh Medical Center, Thomas Starzl Transplant Institute, Pittsburgh, USA
| | | | - G Oldani
- Division of Abdominal Surgery, University Hospitals of Geneva, Geneva, Switzerland
| | - E Palmerini
- Chemotherapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy
| | - M A Pantaleo
- Division of Oncology, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna, Italy
| | - S Patel
- Sarcoma Center, The University of Texas M.D. Anderson Cancer Center, Houston, USA
| | | | - C P Raut
- Department of Surgery, Brigham and Women's Hospital, Boston, USA; Center for Sarcoma and Bone Oncology, Harvard Medical School, Boston, USA; Dana Farber Cancer Center, Harvard Medical School, Boston, USA
| | - V Ravi
- Sarcoma Center, The University of Texas M.D. Anderson Cancer Center, Houston, USA
| | - A R A Razak
- Princess Margaret Cancer Centre and Sinai Healthcare System & Faculty of Medicine, University of Toronto, Toronto, Canada
| | - P Reichardt
- Helios Klinikum Berlin-Buch, Department of Oncology and Palliative Care, Berlin, Germany
| | - B P Rubin
- Robert J. Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, USA
| | - P Rutkowski
- Maria Sklodowska-Curie National Research Institute of Oncology, Department of Soft Tissue/Bone Sarcoma and Melanoma, Warsaw, Poland
| | - A A Safwat
- Aarhus University Hospital, Aarhus, Denmark
| | - C Sangalli
- Department of Radiation Oncology, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - G Sapisochin
- Multi-Organ Transplant and HPB Surgical Oncology, Division of General Surgery, Department of Surgery, University of Toronto, Toronto, Canada
| | - M Sbaraglia
- Department of Pathology, Azienda Ospedaliera Università Padova, Padua, Italy
| | - S Scheipl
- Department of Orthopaedics and Trauma, Medical University of Graz, Graz, Austria
| | | | - D Strauss
- Department of Surgery, The Royal Marsden Hospital and The Institute of Cancer Research, London, UK
| | - S J Strauss
- University College London Hospital, London, UK
| | - K Sundby Hall
- Department of Oncology, Oslo University Hospital, The Norwegian Radium Hospital, Oslo, Norway
| | - W D Tap
- Department of Medicine, Memorial Sloan Kettering Cancer Center and Weill Cornell Medical College, New York, USA
| | - A Trama
- Department of Research, Evaluative Epidemiology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - A Tweddle
- Palliative Care, The Royal Marsden Hospital and The Institute of Cancer Research London
| | - W T A van der Graaf
- Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
| | - M A J Van De Sande
- Department of Orthopedic Surgery Bone and Soft Tissue Tumor Unit, Leiden University Medical Center, Leiden, The Netherlands
| | - W Van Houdt
- Sarcoma and Melanoma Unit, The Netherlands Cancer Institute, Amsterdam, The Netherlands
| | - G van Oortmerssen
- Co-Chair of Sarcoma Patients EuroNet (SPAEN), Woelfersheim, Germany & Chairman of the Dutch organisation for sarcoma patients (Patiëntenplatform Sarcomen), Guest researcher at Leiden University (Leiden Institute for Advanced Computer Science), Leiden University, Leiden, The Netherlands
| | - A J Wagner
- Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, USA
| | - M Wartenberg
- Chair of the Board of Directors of Sarcoma Patients EuroNet (SPAEN), Sarcoma Patients EuroNet (SPAEN), Woelfersheim, Germany
| | - J Wood
- The Royal Marsden NHS Foundation Trust, London, UK
| | - N Zaffaroni
- Molecular Pharmacology Unit, Department of Applied Research and Technological Development, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - C Zimmermann
- Department of Supportive Care, Princess Margaret Cancer Centre and Division of Palliative Medicine, Department of Medicine, University of Toronto, Toronto, Canada
| | - P G Casali
- Adult Mesenchymal Tumor and Rare Cancer Unit, Cancer Medicine Department, Fondazione IRCCS Istituto Nazionale Tumori, Milan, Italy
| | - A P Dei Tos
- Department of Pathology, Azienda Ospedaliera Università Padova, Padua, Italy
| | - A Gronchi
- Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
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22
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Kim SJ, Rhu J, Kim JM, Choi GS, Joh JW. Surgical treatment outcomes of primary hepatic sarcomas: A single-center experience. World J Hepatol 2021; 13:584-594. [PMID: 34131472 PMCID: PMC8173340 DOI: 10.4254/wjh.v13.i5.584] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2021] [Revised: 02/24/2021] [Accepted: 04/25/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Primary hepatic sarcoma is a rare tumor originated from mesenchymal tissue. There are various pathologic types of primary hepatic sarcoma and the treatment outcome of this tumor was usually disappointing. Unlike hepatocellular carcinoma, outcome of primary hepatic sarcoma is not well-known due to it’s rarity. However, with development of medical technology, surgical treatment may lead to better survival.
AIM To investigate the surgical outcomes of primary hepatic sarcoma, we gathered and analyzed the cases of a single institute.
METHODS From August 2001 to September 2016, a total of nine patients were surgically treated for primary hepatic sarcoma after exclusion of cases with open and closure, early loss to follow-up and sarcomatoid hepatocellular carcinoma and sarcomatoid cholangiocellular carcinoma. Baseline characteristics, tumor characteristics such as tumor pathology, size and number, surgical and adjuvant treatments were reviewed. Tumor recurrence, and patient survival were analyzed with retrospective approach.
RESULTS The enrolled participants included five patients with angiosarcoma and four patients with undifferentiated sarcoma. All patients experienced tumor recurrence at a median of 52 post-operative days. Only two patients survived and the 5-year survival rate was 29.6%. One patient with angiosarcoma who received central hepatectomy for primary tumor and received radiofrequency ablation for recurrent tumor still lives for 11 years. One patient with undifferentiated sarcoma received Rt. lobectomy for primary tumor followed by chemotherapy and radiation therapy still lives around 30 mo even though she got additional operation for recurrent tumor. Two patients who received living donor liver transplantation due to angiosarcoma died. Only adjuvant therapy was associated with survival gain (P = 0.002).
CONCLUSION Patients with primary hepatic sarcoma may gain survival benefit with surgical resection followed by adjuvant therapy, even though the outcome remains relatively poor.
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Affiliation(s)
- Sang Jin Kim
- Department of Surgery, Korea University College of Medicine, Seoul, Republic of Korea, Division of Hepatobiliopancreas and Transplant Surgery, Korea University Ansan Hospital, Ansan 15355, South Korea
| | - Jinsoo Rhu
- Department of Surgery, Samsung Medical Center, Seoul 06351, South Korea
| | - Jong Man Kim
- Department of Surgery, Samsung Medical Center, Seoul 06351, South Korea
| | - Gyu Seung Choi
- Department of Surgery, Samsung Medical Center, Seoul 06351, South Korea
| | - Jae-Won Joh
- Department of Surgery, Samsung Medical Center, Seoul 06351, South Korea
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23
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Dogeas E, Mokdad AA, Bhattatiry M, Porembka MR, Polanco PM, Mansour JC, Choti MA, Augustine MM. Tumor Biology Impacts Survival in Surgically Managed Primary Hepatic Vascular Malignancies. J Surg Res 2021; 264:481-489. [PMID: 33857792 DOI: 10.1016/j.jss.2021.02.043] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2020] [Revised: 01/29/2021] [Accepted: 02/27/2021] [Indexed: 12/18/2022]
Abstract
BACKGROUND Hepatic angiosarcoma (AS) and hepatic epithelioid hemangioendothelioma (HEHE) are rare primary hepatic vascular malignancies (PHVM) that remain poorly understood. To guide management, we sought to identify factors and trends predicting survival after surgical intervention using a national database. MATERIALS AND METHODS In a retrospective analysis of the National Cancer Database patients with a diagnosis of PHVM were identified. Clinicopathologic factors were extracted and compared. Overall survival (OS) was estimated and predictors of survival were identified. RESULTS Three hundred ninty patients with AS and 216 with HEHE were identified. Only 16% of AS and 36% of HEHE patients underwent surgery. The median OS for patients who underwent surgical intervention was 97 months, with 5-year OS of 30% for AS versus 69% for HEHE patients (P< 0.001). Tumor biology strongly impacted OS, with AS histology (Hazard Ratio [HR] of 3.61 [1.55-8.42]), moderate/poor tumor differentiation (HR = 3.86 [1.03-14.46]) and tumor size (HR = 1.01 [1.00-1.01]) conferring worse prognosis. The presence of metastatic disease in the surgically managed cohort (HR = 5.22 [2.01-13.57]) and involved surgical margins (HR = 3.87 [1.59-9.42]), were independently associated with worse survival. CONCLUSIONS In this national cohort of PHVM, tumor biology, in the form of angiosarcoma histology, tumor differentiation and tumor size, was strongly associated with worse survival after surgery. Additionally, residual tumor burden after resection, in the form of positive surgical margins or the presence of metastasis, was also negatively associated with survival. Long-term clinical outcomes remain poor for patients with the above high-risk features, emphasizing the need to develop effective forms of adjuvant systemic therapies for this group of malignancies.
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Affiliation(s)
- Epameinondas Dogeas
- Department of Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania
| | - Ali A Mokdad
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Mitu Bhattatiry
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Matthew R Porembka
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Patricio M Polanco
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - John C Mansour
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas
| | - Michael A Choti
- Department of Surgery, Banner MD Anderson Cancer Center, Phoenix, Arizona
| | - Mathew M Augustine
- Department of Surgery, University of Texas Southwestern Medical Center, Dallas, Texas; North Texas Veterans Affairs Medical Center, Dallas, Texas.
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24
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Jiang L, Xie L, Li G, Xie H, Fang Z, Cai X, Chen Y. Clinical characteristics and surgical treatments of primary hepatic angiosarcoma. BMC Gastroenterol 2021; 21:156. [PMID: 33827427 PMCID: PMC8028144 DOI: 10.1186/s12876-021-01743-3] [Citation(s) in RCA: 12] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2021] [Accepted: 03/30/2021] [Indexed: 12/26/2022] Open
Abstract
PURPOSE Primary hepatic angiosarcoma is a very rare and highly malignant tumor with poor prognosis. It is difficult to diagnose because of the lack of typical clinical features, and the treatment protocols for PHA are also not clear. Therefore, this study wants to find out the clinical characteristics and surgical treatments of primary hepatic angiosarcoma. METHODS Among 8990 patients diagnosed with primary malignant tumor of the liver from January 2000 to December 2019 in our hospital, only four patients were diagnosed with primary hepatic angiosarcoma. The demographics, clinical manifestation, past history, serology test results, MRI features, pathology, treatment modality and prognosis of four patients were collected and analyzed. RESULTS Three of four patients had no clinical symptoms, while one patient's symptom was abdominal pain. The levels of tumor markers of all four patients were within the normal reference range and serological tests were negative for hepatitis B and C virus. The MRI imaging findings of all four patients were mixed mass with highly disordered vascular characteristics. All four patients were misdiagnosed preoperatively. One patient who underwent hepatic lobectomy was still alive for about 18 months after surgery. One patient who underwent hepatic lobectomy has survived for only 6 months due to severe pneumonia. The other two patients who received transarterial chemoembolization survived 16 months and 11 months respectively. CONCLUSION The clinical symptoms of primary hepatic angiosarcoma are not typical, and primary hepatic angiosarcoma is easily misdiagnosed. The typical imaging manifestations are structural disorder and heterogeneous tumor. Hepatic lobectomy and transarterial chemoembolization may be important surgical treatments to improve the prognosis of patients.
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Affiliation(s)
- Lei Jiang
- Department of Hepatobiliary Surgery and Fujian Institute of Hepatobiliary Surgery, Fujian Medical University Union Hospital, NO. 29, Xinquan Road, Fuzhou City, Fujian Province, China.,Key Laboratory of The Ministry of Education for Gastrointestinal Cancer, Fujian Medical University, Fuzhou, 350108, Fujian, China
| | - Lijun Xie
- Department of Ultrasonic Image, The First Affiliated Hospital of Fujian Medical University, Fuzhou, 350005, Fujian, China
| | - Ge Li
- Department of Hepatobiliary Surgery and Fujian Institute of Hepatobiliary Surgery, Fujian Medical University Union Hospital, NO. 29, Xinquan Road, Fuzhou City, Fujian Province, China.,Key Laboratory of The Ministry of Education for Gastrointestinal Cancer, Fujian Medical University, Fuzhou, 350108, Fujian, China
| | - Hang Xie
- Department of Intervention Therapy, Fujian Medical University Union Hospital, Fujian, 350001, China
| | - Zhao Fang
- Department of Hepatobiliary Surgery and Fujian Institute of Hepatobiliary Surgery, Fujian Medical University Union Hospital, NO. 29, Xinquan Road, Fuzhou City, Fujian Province, China
| | - Xinran Cai
- Department of Hepatobiliary Surgery and Fujian Institute of Hepatobiliary Surgery, Fujian Medical University Union Hospital, NO. 29, Xinquan Road, Fuzhou City, Fujian Province, China. .,Key Laboratory of The Ministry of Education for Gastrointestinal Cancer, Fujian Medical University, Fuzhou, 350108, Fujian, China.
| | - Yanling Chen
- Department of Hepatobiliary Surgery and Fujian Institute of Hepatobiliary Surgery, Fujian Medical University Union Hospital, NO. 29, Xinquan Road, Fuzhou City, Fujian Province, China. .,Key Laboratory of The Ministry of Education for Gastrointestinal Cancer, Fujian Medical University, Fuzhou, 350108, Fujian, China.
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25
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Gigante E, Paradis V, Ronot M, Cauchy F, Soubrane O, Ganne-Carrié N, Nault JC. New insights into the pathophysiology and clinical care of rare primary liver cancers. JHEP Rep 2021; 3:100174. [PMID: 33205035 PMCID: PMC7653076 DOI: 10.1016/j.jhepr.2020.100174] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2020] [Revised: 08/06/2020] [Accepted: 08/10/2020] [Indexed: 02/07/2023] Open
Abstract
Hepatocholangiocarcinoma, fibrolamellar carcinoma, hepatic haemangioendothelioma and hepatic angiosarcoma represent less than 5% of primary liver cancers. Fibrolamellar carcinoma and hepatic haemangioendothelioma are driven by unique somatic genetic alterations (DNAJB1-PRKCA and CAMTA1-WWTR1 fusions, respectively), while the pathogenesis of hepatocholangiocarcinoma remains more complex, as suggested by its histological diversity. Histology is the gold standard for diagnosis, which remains challenging even in an expert centre because of the low incidences of these liver cancers. Resection, when feasible, is the cornerstone of treatment, together with liver transplantation for hepatic haemangioendothelioma. The role of locoregional therapies and systemic treatments remains poorly studied. In this review, we aim to describe the recent advances in terms of diagnosis and clinical management of these rare primary liver cancers.
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Key Words
- 5-FU, 5-Fluorouracil
- AFP, alpha-fetoprotein
- APHE, arterial phase hyperenhancement
- CA19-9, carbohydrate antigen 19-9
- CCA, cholangiocarcinoma
- CEUS, contrast-enhanced ultrasound
- CK, cytokeratin
- CLC, cholangiolocellular carcinoma
- EpCAM, epithelial cell adhesion molecule
- FISH, fluorescence in situ hybridisation
- FLC, fibrolamellar carcinoma
- Fibrolamellar carcinoma
- HAS, hepatic angiosarcoma
- HCC, hepatocellular carcinoma
- HEH, hepatic epithelioid haemangioendothelioma
- HepPar1, hepatocyte specific antigen antibody
- Hepatic angiosarcoma
- Hepatic hemangioendothelioma
- Hepatocellular carcinoma
- Hepatocholangiocarcinoma
- IHC, immunohistochemistry
- LI-RADS, liver imaging reporting and data system
- LT, liver transplantation
- Mixed tumor
- RT-PCR, reverse transcription PCR
- SIRT, selective internal radiation therapy
- TACE, transarterial chemoembolisation
- WHO, World Health Organization
- cHCC-CCA, combined hepatocholangiocarcinoma
- iCCA, intrahepatic cholangiocarcinoma
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Affiliation(s)
- Elia Gigante
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
| | - Valérie Paradis
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service d'anatomie pathologique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Maxime Ronot
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de radiologie, Hôpital Beaujon, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - François Cauchy
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de chirurgie hépato-bilio-pancréatique et transplantation hépatique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Olivier Soubrane
- Centre de recherche sur l’inflammation, Inserm, Université de Paris, INSERM UMR 1149 « De l'inflammation au cancer », Paris, France
- Service de chirurgie hépato-bilio-pancréatique et transplantation hépatique, Hôpitaux Universitaires Paris-Nord-Val-de-Seine, Assistance-Publique Hôpitaux de Paris, Clichy, France
- Université de Paris, Paris, France
| | - Nathalie Ganne-Carrié
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
- Centre de Recherche des Cordeliers, Inserm, Sorbonne Université, Université Paris, INSERM UMR 1138, Functional Genomics of Solid Tumors, F-75006, Paris, France
| | - Jean-Charles Nault
- Service d’hépatologie, Hôpital Avicenne, Hôpitaux Universitaires Paris-Seine-Saint-Denis, Assistance-Publique Hôpitaux de Paris, Bobigny, France
- Unité de Formation et de Recherche Santé Médecine et Biologie Humaine, Université Paris 13, Paris, France
- Centre de Recherche des Cordeliers, Inserm, Sorbonne Université, Université Paris, INSERM UMR 1138, Functional Genomics of Solid Tumors, F-75006, Paris, France
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26
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Martins ACDEA, Costa Neto DCDA, Silva JÚDDEME, Moraes YM, LeÃo CS, Martins C. Adult primary liver sarcoma: systematic review. ACTA ACUST UNITED AC 2020; 47:e20202647. [PMID: 33263653 DOI: 10.1590/0100-6991e-20202647] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/30/2020] [Accepted: 06/29/2020] [Indexed: 12/28/2022]
Abstract
INTRODUCTION primary liver sarcoma is a rare type of tumor, more common in children. Among adults, it represents a spectrum of neoplasms with reserved prognosis. There is no consensus on the treatment of choice of these lesions, justifying a systematic review of the literature on treatment options, prognostic factors, and survival. MATERIAL/METHODS a systematic review of articles published in Pubmed, Medline, LiLacs e SciElo, from 1966 to March/2019, presenting the keywords: primary-liver-sarcoma and primary-hepatic-sarcoma was undertaken. Studies including patients older than 18 years, and published in English, Portuguese and Spanish were included. Case reports, metastatic tumors and multiple oncologic diagnosis were excluded. The initial search listed 1,318 articles. 1,206 did not meet the inclusion criteria. After reviewing 112 eligible articles, 15 were selected (14 case series and 1 retrospective-cohort). RESULTS proposed treatment modalities for primary liver sarcoma included surgery and/or chemotherapy and/or radiotherapy or liver transplantation. The most common histological types were angiosarcoma (32%), leiomyosarcoma (29%), epithelioid hemangioendothelioma (15%) and embryonal sarcoma (7%). Histology, degree of differentiation and R0 resection were mentioned positive prognostic factors. Median survival ranged from two to 23 months. Five-year survival rate varied from 0% to 64%, on average 21%. CONCLUSION surgical resection (R0 resection) is the main treatment for primary liver sarcomas. Development of effective systemic therapies are required to improve prognosis of patients harboring this type of tumor.
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Affiliation(s)
- Antonio Cavalcanti DE Albuquerque Martins
- - Instituto de Medicina Integral Prof. Fernando Figueira, Departamento de Cirurgia Geral - Recife - PE - Brasil.,- Faculdade Pernambucana de Saúde - FPS/IMIP, Curso de Medicina - Recife - PE - Brasil
| | | | | | - Ygor Monteiro Moraes
- - Faculdade Pernambucana de Saúde - FPS/IMIP, Curso de Medicina - Recife - PE - Brasil
| | - Cristiano Souza LeÃo
- - Instituto de Medicina Integral Prof. Fernando Figueira, Departamento de Cirurgia Geral - Recife - PE - Brasil.,- Faculdade Pernambucana de Saúde - FPS/IMIP, Curso de Medicina - Recife - PE - Brasil
| | - Carolina Martins
- - Hospital Metropolitano Oeste Pelópidas Silveira (HPS) - SUS-PE/IMIP, Diretoria de Ensino e Pesquisa - Recife - PE - Brasil.,- Universidade Federal de Pernambuco - UFPE, Coordenação de Pequisa - Centro de Ciências Médicas - Recife - PE - Brasil
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27
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Aziz H, Hanna K, Saif MW, Rauf MA, Genyk Y, Sheikh MR. Hepatectomy for Breast Cancer Metastasis and Sarcoma are more likely to have Adverse Outcomes than Hepatectomy for Primary Hepatocellular Cancer or for Colorectal Metastasis. CANCER MEDICINE JOURNAL 2020; 3:6-12. [PMID: 33196059 PMCID: PMC7664114] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Subscribe] [Scholar Register] [Indexed: 06/11/2023]
Abstract
INTRODUCTION Outcomes for hepatectomy for breast cancer metastasis and sarcomatous disease processes are not well defined in literature. We sought to use a national database to identify outcomes in these patients compared to subset of patients more well studied in literature - primary Hepatocellular cancer patients and patients with colorectal metastasis. METHODS We identified patients undergoing major hepatectomy (≥ 3 segments) for primary hepatocellular cancer (HCC), sarcoma metastasis, breast cancer metastasis, and colorectal metastasis using NSQIP database. The Primary outcome measure was 30-day mortality. Secondary outcome measures were 30-day readmission and complication rates. RESULTS A total of 5580 patients underwent major hepatectomy during the study period. Patients who underwent hepatectomy for breast cancer metastasis had higher incidence of in-hospital complications (37%) compared to sarcoma (29%), colon (26%), and HCC patients (24%) and 30-days readmission rate (37% vs. 29% - sarcoma vs. 26% - colon vs. 25% HCC). There was no difference in 30-days mortality among the groups. CONCLUSION Patients undergoing major hepatectomies for breast cancer metastasis and sarcoma are more likely to have adverse outcomes than compared to their counterparts. This difference highlights the lack of experience in managing breast cancer and sarcoma with metastatic disease to the liver. This also highlights the difference in tumor biology among all the lesions we studied. An extensive discussion should take place when dealing with breast and sarcoma lesions in the liver because of these outcomes.
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Affiliation(s)
- Hassan Aziz
- Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
| | - Kamil Hanna
- Westchester Medical Center, Valhalla, NY, USA
| | - Muhammad Wasif Saif
- Northwell Health Cancer Institute, Zucker School of Medicine and Feinstein Institutes of Research, Manhasset, NY, USA
| | | | - Yuri Genyk
- Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
| | - Mohd Raashid Sheikh
- Department of Surgery, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA
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28
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LeGout JD, Bailey RE, Bolan CW, Bowman AW, Chen F, Cernigliaro JG, Alexander LF. Multimodality Imaging of Abdominopelvic Tumors with Venous Invasion. Radiographics 2020; 40:2098-2116. [PMID: 33064623 DOI: 10.1148/rg.2020200047] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
A broad range of abdominal and pelvic tumors can manifest with or develop intraluminal venous invasion. Imaging features at cross-sectional modalities and contrast-enhanced US that allow differentiation of tumor extension within veins from bland thrombus include the expansile nature of tumor thrombus and attenuation and enhancement similar to those of the primary tumor. Venous invasion is a distinctive feature of hepatocellular carcinoma and renal cell carcinoma with known prognostic and treatment implications; however, this finding remains an underrecognized characteristic of multiple other malignancies-including cholangiocarcinoma, adrenocortical carcinoma, pancreatic neuroendocrine tumor, and primary venous leiomyosarcoma-and can be a feature of benign tumors such as renal angiomyolipoma and uterine leiomyomatosis. Recognition of tumor venous invasion at imaging has clinical significance and management implications for a range of abdominal and pelvic tumors. For example, portal vein invasion is a strong negative prognostic indicator in patients with hepatocellular carcinoma. In patients with rectal cancer, diagnosis of extramural venous invasion helps predict local and distant recurrence and is associated with worse survival. The authors present venous invasion by vascular distribution and organ of primary tumor origin with review of typical imaging features. Common pitfalls and mimics of neoplastic thrombus, including artifacts and anatomic variants, are described to help differentiate these findings from tumor in vein. By accurately diagnosing tumor venous invasion, especially in tumors where its presence may not be a typical feature, radiologists can help referring clinicians develop the best treatment strategies for their patients. ©RSNA, 2020.
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Affiliation(s)
- Jordan D LeGout
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Ryan E Bailey
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Candice W Bolan
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Andrew W Bowman
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Frank Chen
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Joseph G Cernigliaro
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
| | - Lauren F Alexander
- From the Department of Radiology, Mayo Clinic Florida, 4500 San Pablo Rd, Jacksonville, FL 32224
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29
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Terasaki F, Yamamoto Y, Sugiura T, Okamura Y, Ito T, Ashida R, Ohgi K, Uesaka K. Laparoscopic repeat liver resection for hepatic epithelioid hemangioendothelioma. Surg Case Rep 2020; 6:254. [PMID: 33001327 PMCID: PMC7530154 DOI: 10.1186/s40792-020-01036-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/21/2020] [Accepted: 09/25/2020] [Indexed: 01/23/2023] Open
Abstract
BACKGROUND Optimal treatment for patients with hepatic epithelioid hemangioendothelioma (HEHE) remains unclear. Laparoscopic repeat liver resection (LR) is a minimally invasive and potentially effective surgical option for multiple HEHEs. CASE PRESENTATION A 42-year-old woman with no relevant history was admitted for multiple liver tumors. Six tumors were observed on T2-weighted magnetic resonance imaging (MRI) including one in S2, two in S3, two in S7, and one in S8. Pathological evaluation of percutaneous tumor biopsy tissue suggested a diagnosis of HEHE and laparoscopic LR was planned. The procedure began with partial resection of S7 and partial resection of S8 and left lateral sectionectomy were performed. Another tumor was found intraoperatively on the surface of S6, necessitating removal by partial resection. Pathological evaluation of the resected tumor tissue from all seven tumors concurred with that of the preoperative biopsy. The patient was discharged on postoperative day 6 without any complications. A follow-up MRI 15 months after the primary surgery revealed one tumor each in S4, S6, and S8. Laparoscopic repeat LR was performed. The patient was discharged on postoperative day 5 without any complications. All three recurrent tumors were pathologically confirmed as HEHEs. CONCLUSIONS We successfully treated primary and recurrent HEHEs with laparoscopic LR, which is a reasonable minimally invasive procedure considering the possibility of multiple courses of liver surgery in patients with HEHE.
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Affiliation(s)
- Fumihiro Terasaki
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Yusuke Yamamoto
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan.
| | - Teiichi Sugiura
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Yukiyasu Okamura
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Takaaki Ito
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Ryo Ashida
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Katsuhisa Ohgi
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
| | - Katsuhiko Uesaka
- Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimonagakubo, Nagaizumi-cho, Sunto-gun, Shizuoka, 411-8777, Japan
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30
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Wu V, McArthur MA, Allen A, Manon L, Xie KL. Rare primary hepatic malignancies: A case-based review. Clin Imaging 2020; 69:196-204. [PMID: 32919206 DOI: 10.1016/j.clinimag.2020.08.004] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2020] [Revised: 07/07/2020] [Accepted: 08/10/2020] [Indexed: 11/29/2022]
Abstract
The two most common primary liver malignancies that radiologists encounter in clinical practice are hepatocellular carcinoma (HCC) and intrahepatic cholangiocarcinoma (ICC). However, there are other less common primary hepatic malignancies that radiologists should be aware of. The correct radiographic and pathologic diagnosis of these entities have important treatment and prognostic implications. In this paper, we review a series of five cases that we have encountered in clinical practice at our institution that were initially thought to be HCC or ICC, but turned out to be a rarer primary hepatic malignancy. We will review the radiographic and pathologic characteristics of each of these rare primary hepatic malignancies as well as discuss the prognosis and treatment for each.
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Affiliation(s)
- Victoria Wu
- University of Illinois in Chicago, 1740 W. Taylor Street, 2511 UIH, Chicago, IL 60612, USA.
| | - Mark A McArthur
- University of Illinois in Chicago, 1740 W. Taylor Street, 2511 UIH, Chicago, IL 60612, USA
| | - Amanda Allen
- University of Illinois in Chicago, 1740 W. Taylor Street, 2511 UIH, Chicago, IL 60612, USA
| | - Luis Manon
- University of Illinois in Chicago, 1740 W. Taylor Street, 2511 UIH, Chicago, IL 60612, USA.
| | - Karen L Xie
- University of Illinois in Chicago, 1740 W. Taylor Street, 2511 UIH, Chicago, IL 60612, USA.
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31
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Kyriazoglou A, Koutsoukos K, Zagouri F, Liontos M, Dimitriadis E, Tiniakos D, Dimopoulos MA. Metastatic Hepatic Epithelioid Hemangioendothelioma Treated with Olaratumab: A Falling Star Rising? Ther Clin Risk Manag 2020; 16:141-146. [PMID: 32161464 PMCID: PMC7051805 DOI: 10.2147/tcrm.s220804] [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: 06/25/2019] [Accepted: 11/12/2019] [Indexed: 11/23/2022] Open
Abstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular malignant tumor with indolent course. Liver transplantation for local disease is the treatment of choice. In the metastatic setting there is no consensus regarding the appropriate systemic treatment. We present two cases of metastatic hepatic epithelioid hemangioendothelioma (hEHE) treated with the combination of Doxorubicin and Olaratumab. Both patients showed Stable Disease (SD) as a response, after the completion of six cycles of this combination therapy.
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Affiliation(s)
| | | | - Flora Zagouri
- Department of Clinical Therapeutics, General Hospital Alexandra, Athens, Greece
| | - Michalis Liontos
- Department of Clinical Therapeutics, General Hospital Alexandra, Athens, Greece
| | | | - Dina Tiniakos
- Department of Pathology Aretaion Hospital, National & Kapodistrian University of Athens, Athens, Greece.,Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
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32
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Esposito F, Lim C, Baranes L, Salloum C, Feray C, Calderaro J, Azoulay D. Primary leiomyosarcoma of the liver: Two new cases and a systematic review. Ann Hepatobiliary Pancreat Surg 2020; 24:63-67. [PMID: 32181431 PMCID: PMC7061050 DOI: 10.14701/ahbps.2020.24.1.63] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/30/2019] [Revised: 05/05/2019] [Accepted: 05/09/2019] [Indexed: 12/12/2022] Open
Abstract
Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. Clinical presentation and imaging features are non-specific and can mimick the most frequent primary liver tumors namely hepatocellular carcinoma and intrahepatic cholangiocarcinoma. We report here two cases of PHL including one from the portal vein. The literature was searched for studies reporting cases of PHL reported from 2011 and 2019. The two patients were operated with R0 resection. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. Surgery remains the mainstay of the management of PHL. R0 resection is the main prognostic factor. Our literature search identified 16 additional cases from 12 reports. Preoperative diagnosis of PHL needs a high degree of suspicion due to atypical clinical presentation and non-specific imaging features. Surgery is the mainstay of the management of PHL. R0 resection is the main prognostic factor.
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Affiliation(s)
| | - Chetana Lim
- Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Pitié-Salpêtrière Hospital, Paris, France
| | | | - Chady Salloum
- Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Paul Brousse Hospital, Villejuif, France
| | - Cyrille Feray
- Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Paul Brousse Hospital, Villejuif, France
| | | | - Daniel Azoulay
- Department of Hepatobiliary and Pancreatic Surgery and Liver Transplantation, Paul Brousse Hospital, Villejuif, France.,Department of Hepatobiliary and Pancreatic Surgery and Transplantation, Sheba Medical Center, Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
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33
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Yuan WH, Li AFY, Hsu HC, Hu YS, Lee RC. Initial clinical radiological findings and staging to predict prognosis of primary hepatic angiosarcoma: A retrospective analysis. PLoS One 2019; 14:e0225043. [PMID: 31710641 PMCID: PMC6844487 DOI: 10.1371/journal.pone.0225043] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/03/2019] [Accepted: 10/27/2019] [Indexed: 12/13/2022] Open
Abstract
OBJECTIVE Primary hepatic angiosarcoma (PHA) is extremely rare and most patients die within 12 months of diagnosis. The object of the study is to determine the association of initial clinical-radiological features and staging with outcomes in patients with PHA. METHODS The medical records of adult patients with PHA were retrieved from an electronic medical record database and a pathology database and retrospectively reviewed. During 10 years, 22 eligible patients were included. Data extracted focused on the information before the first formal treatment with a pathological proof, including demographic characteristics, medical history, laboratory data, preliminary images, histopathological records, treatment, and follow-up survival period. Two radiologists blindly re-analyzed preliminary images of all 22 patients together and recorded tumor features and imaging stage based on the American Joint Committee on Cancer (AJCC) 8th edition tumor-node-metastasis (TNM) Staging System for hepatocellular carcinoma. A radiologist compiled the initial clinical data and preliminary image stage to analyze the association with patients' survival outcome. RESULTS Higher aspartate aminotransferase (AST), higher total bilirubin (TB), lower albumin (ALB), longer prothrombin time (PT) and lower platelet count of serum relative to the normal reference range were more common in patients who survived ≤ 90 days (all P < 0.05). Overall survival was much better in patients with single PHA than in those with other tumor patterns of multiple PHA (all P < 0.05). Overall survival determined by preliminary imaging showed significant differences between stage I and stage III (P = 0.044), stage I and stage IV (P = 0.011), and stage III and IV (P = 0.047). No patients were at stage II. CONCLUSIONS Initial serum levels of ALT, TB, ALB, and PT, platelet count, single mass in liver, and preliminary imaging staging could help predict survival outcomes of patients with PHA.
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Affiliation(s)
- Wei-Hsin Yuan
- Division of Radiology, Taipei Municipal Gan-Dau Hospital (managed by Taipei Veterans General Hospital), Taipei, Taiwan, Republic of China
- School of Medicine, National Yang Ming University, Taipei, Taiwan, Republic of China
- Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
- * E-mail: , (WHY); (RCL)
| | - Anna Fen-Yau Li
- School of Medicine, National Yang Ming University, Taipei, Taiwan, Republic of China
- Department of Pathology, Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
| | - Hui-Chen Hsu
- Department of Medical Imaging, Taiwan Adventist Hospital, Taipei, Taiwan, Republic of China
| | - Yong-Sin Hu
- School of Medicine, National Yang Ming University, Taipei, Taiwan, Republic of China
- Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
| | - Rheun-Chuan Lee
- School of Medicine, National Yang Ming University, Taipei, Taiwan, Republic of China
- Department of Radiology, Taipei Veterans General Hospital, Taipei, Taiwan, Republic of China
- * E-mail: , (WHY); (RCL)
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Recent advances in liver transplantation for cancer: The future of transplant oncology. JHEP Rep 2019; 1:377-391. [PMID: 32039389 PMCID: PMC7005652 DOI: 10.1016/j.jhepr.2019.07.004] [Citation(s) in RCA: 20] [Impact Index Per Article: 3.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2019] [Revised: 07/15/2019] [Accepted: 07/16/2019] [Indexed: 02/06/2023] Open
Abstract
Liver transplantation is widely indicated as a curative treatment for selected patients with hepatocellular carcinoma. However, with recent therapeutic advances, as well as efforts to increase the donor pool, liver transplantation has been carefully expanded to patients with other primary or secondary malignancies in the liver. Cholangiocarcinoma, colorectal and neuroendocrine liver metastases, and hepatic epithelioid haemangioendothelioma are amongst the most relevant new indications. In this review we discuss the fundamental concepts of this ambitious undertaking, as well as the newest indications for liver transplantation, with a special focus on future perspectives within the recently established concept of transplant oncology.
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35
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Wilson GC, Lluis N, Nalesnik MA, Nassar A, Serrano T, Ramos E, Torbenson M, Asbun HJ, Geller DA. Hepatic Angiosarcoma: A Multi-institutional, International Experience with 44 Cases. Ann Surg Oncol 2019; 26:576-582. [DOI: 10.1245/s10434-018-7062-9] [Citation(s) in RCA: 27] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2018] [Indexed: 08/30/2023]
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