Liver Angiosarcoma: Rare tumour associated with a poor prognosis, literature review and case report
نویسندگان
چکیده
INTRODUCTION Liver angiosarcoma is a very uncommon tumour of mesenchymal origin, representing between 0.1-2% of all primary tumours of the liver, affecting mainly men in their sixth or seventh decade of life, with a high mortality in the first years (Chaudhary et al., 2015). Literature reports of its surgical treatment vary from a total or partial hepatectomy with or without liver transplant. PRESENTATION OF CASE A 37year old male, with a 7year history of a fatty liver, was found to have a 12cm diameter tumour in a cirrhotic liver, during an abdominal Computed Tomography (CT) scan. Patient was asymptomatic with negative tumour markers, yet tumour liver biopsy revealed a Liver Angiosarcoma with positive immunohistochemistry for neoplastic cells CD31 and CD34. Patient was deemed candidate for a partial hepatectomy of the affected liver segments which was done without complications and no evidence of other tumour lesions was found during surgery. Patient continued oncologic management with ongoing chemotherapy. DISCUSION Liver Angiosarcoma, although rare, persists with a high mortality due to its aggressive nature. Never the less liver transplantation, although proven to be an effective treatment for many pathologies that culminate in liver failure, fails to improve patients' survival and prognosis, when compared to partial hepatectomy as surgical management to for liver Angiosarcoma, CONCLUSION: Partial hepatectomy as surgical management, followed by adjuvant therapy, for Liver Angiosarcoma continues to prove favourable results and prognosis compared to Liver Transplantation.
منابع مشابه
Metastatic Angiosarcoma of Liver: A Case Report Authors
1 Associate Professor, 2 Post-graduate trainees, 3 Professor Department of Surgery, Regional Institute of Medical Sciences, Imphal, Manipur, India Email: [email protected] Corresponding Author Dr Lalsangzuala Tochhawng PG Student, Department of Surgery, Regional Institute of Medical Sciences, Imphal, Manipur, INDIA Abstract Angiosarcoma is a rare malignant tumour of endothelial origin with a...
متن کاملOvarian Small Cell Carcinoma: A Rare Case Report and Review of Literature
Ovarian small cell carcinoma is a rare and highly malignant tumor with poor prognosis. It usually presents in younger females with features of hypercalcemia. The exact histogenesis of the tumor is unclear and it may present as an undifferentiated tumor. In such cases, immunohistochemistry (IHC) plays an important role to confirm the diagnosis. Limited treatment options are available and mainly ...
متن کاملPrimary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature
Angiosarcoma is a rare disease with a poor prognosis; significantly, patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare. This article describes the case of a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which had increased in severity 2 weeks prior to presentation. After a complicated diagnostic and therapeuti...
متن کاملPrimary Small Cell Carcinoma of the Esophagus (PSCEC) Associated with Paraneoplastic Sweating Syndrome: A Case Report and Literature Review
Introduction: Primary small cell carcinoma of theesophagus (PSCEC) associated with paraneoplastic sweating syndrome is a rare disease characterized with rapid growth rate, metastasis at the time of diagnosis, and poor prognosis. The lung is the most common site for small cell carcinoma but this malignancy includes 0.1% to 1% of all gastrointestinal and 0.8% to 2.7% of esophageal malignancies. ...
متن کاملFulminant Hepatic Failure Secondary to Primary Hepatic Angiosarcoma
Background. Hepatic angiosarcoma is a rare and aggressive tumor that often presents at an advanced stage with nonspecific symptoms. Objective. To report a case of primary hepatic angiosarcoma in an otherwise healthy man with normal liver function tests two months prior to presenting with a short period of jaundice that progressed to fulminant hepatic failure. Methods. Case report and review of ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 28 شماره
صفحات -
تاریخ انتشار 2016