Hepatic epithelioid hemangioendothelioma: Resection or transplantation, which and when?

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an unp redictable course and prognosis, The aim of this study is to describe our e xperience with liver resection, as well as transplantation, in the treatmen t of this tumor. We retrospectively analyzed the clinical features, patholo gical findings, and postoperative results in a series of 11 patients presen ting between 1990 and 1998, Five patients (45%) presented with abdominal pa in, 3 patients (27%) with jaundice and ascites, and the rest were asymptoma tic. Computed tomography or magnetic resonance imaging showed localized les ions in 2 patients (18%) and multifocal disease in the others, Seven patien ts (64%) had extrahepatic lesions, detected either by preoperative imaging or discovered at exploration, Two resections of apparently localized lesion s were followed by rapid and aggressive recurrence. Five patients were trea ted with transplantation, including 1 patient who had previously undergone resection, Of these 5 patients, 2 patients are currently free of detectable disease, 1 patient who had severe ascites and jaundice is now asymptomatic with stable extrahepatic lesions, and 2 patients (including 1 who had prev iously undergone a resection) died of tumor recurrence. One patient with ad vanced tumor died while waiting for transplantation. The remaining 4 patien ts are free of symptoms and have stable hepatic and extrahepatic disease. H EHE is nearly always multifocal, and our results with resection were dismal . Because of the unpredictable nature of the tumor, the indications for tra nsplantation in patients without liver-related symptoms should be carefully evaluated, Nevertheless, extrahepatic disease should not be an absolute co ntraindication for liver transplantation in patients with severe liver dysf unction. Copyright (C) 1999 by the American Association for the Study of Li ver Diseases.