Dabashish Patowary1
1. Assistant Professor, Department of Radiotherapy, North East Cancer Hospital, Sylhet, Bangladesh
Abstract
Epithelioid hemangioendothelioma (HEHE) is a aggressive vascular tumor of the hemangioendothelioma family. These cases are usually but may present with abdominal pain, hepatomegaly and weight loss. Radiologically, HEHE presents with a multifocal nodular pattern & extensive mass of coalesced lesion in early & advanced stage respectively. Macroscopically, HEHE lesions are usually multifocal with ill-defined nodules scattered throughout the liver & microscopically the tumor cells resembles signet ring-like structures. The definite diagnosis is found by immunohistochemistry for endothelial markers. We have a case of HEHE for a patient of 56 years old admitted in Apollo hospitals Chennai with complaints of abdominal pain for the last 3 months. Multiple enlarged lymph nodes noted at peri-pancreatic and porta hepatic region along with few pre-vertebral enlarged lymph nodes at lower thoracic region. Liver (needle) biopsy showed sclerotic lesion with patchy necrosis & multifocal atypical cellular proliferation, favoring neoplasm. However, the tumor was pathologically diagnosed as Hepatic Epithelioid hemangioendothelioma (HEHE) based on histological & immunohistochemical findings. There is no standard strategy for the treatment and follow up of HEHE because of its rarity & variable clinical course. Currently, the treatment of HEHE includes liver transplantation (44.8%), chemotherapy or radiotherapy (21%), liver resection (9.4%) & no treatment (24.8%). The prognostic factors of HEHE remain unclear as well.
Key Words: Epithelioid hemangioendothelioma, Endothelial marker, Liver.