Abstract
BackgroundHepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Here, we describe the clinicopathological features of hepatic epithelioid hemangioendothelioma cases seen in our practice and present a detailed review of the published literature.MethodsAll cases of hepatic epithelioid hemangioendothelioma diagnosed in Department of Pathology and Laboratory Medicine, Aga Khan University Hospital between January 1, 2006 and December 31, 2019 were included in the study. Slides were reviewed and follow up was obtained.ResultsSeven cases were reported during the study period. There were 4 females and 3 males. Age range was 20 to 77 years, mean age was 45 years. Three patients presented with right upper abdominal pain; 1 patient presented with jaundice while 3 patients were asymptomatic. In all 7 cases, lesions were identified on imaging studies. In 5 cases, liver lesions were multifocal. Clinical differential diagnosis in all cases was metastatic carcinoma and multifocal hepatocellular carcinoma. Liver function tests were normal in 5 cases. In 1 patient, tumor had already metastasized to the right lung. On histological examination of liver core biopsies performed in all 7 cases, classic histological features of epithelioid hemangioendothelioma were seen. Tumor cells expressed positivity for vascular markers (CD 34, CD31 and ERG) and were negative for cytokeratins, Hep par 1 and Glypican 3. Surgical resection was not performed in any of the 7 cases and all patients were treated by chemotherapy. Follow up was available in 5 cases. Of these, 3 patients died of disease and another patient was alive with metastases in both lungs, omentum and colon.ConclusionClinicopathological features of the 7 cases in our series and detailed review of published literature is presented. Prognosis was bad in our cases most likely due to fact that surgical resection could not be performed in any of the cases owing to lack of surgical expertise for liver tumor surgery in most parts of the country.
Highlights
Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally
The aim of the present study is to present the clinicopathological features of cases of primary hepatic Epitheloid hemangioendothelioma (EHE) diagnosed in our practice and to present a detailed review of the published literature on these rare neoplasms
Clinical suspicion of metastatic tumor and multifocal hepatocellular carcinoma (HCC) was raised in these cases
Summary
Hepatic epithelioid hemangioendothelioma is an extremely rare malignant vascular tumor which is often multifocal and, in many cases, discovered incidentally. Hepatic epithelioid hemangioendothelioma (EHE) is a very rare malignant vascular tumor composed of epithelioid and histiocytoid endothelial cells in a myxohyaline or fibrous stroma. It may occur in the lungs, soft tissue and bone. Over 75% cases are multifocal often involving the right and left lobes of the liver It is often discovered as an incidental finding on radiological examination but sometimes may present with abdominal pain, weight loss or ascites. YAP 1 –TFE3 fusions have been identified These tumors have a variable clinical course; some patients have progressive disease while others have more indolent and stable course. It needs to be emphasized that owing to the rarity of these tumors, there are still no consensus treatment guidelines or protocols and these tumors are usually treated by radical surgical resection or liver transplant and/ or chemotherapy [2,3,4,5,6,7]
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