Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor with an unpredictable course and prognosis. The aim of this study is to describe our experience with liver resection, as well as transplantation, in the treatment of this tumor. We retrospectively analyzed the clinical features, pathological findings, and postoperative results in a series of 11 patients presenting between 1990 and 1998. Five patients (45%) presented with abdominal pain, 3 patients (27%) with jaundice and ascites, and the rest were asymptomatic. Computed tomography or magnetic resonance imaging showed localized lesions in 2 patients (18%) and multifocal disease in the others. Seven patients (64%) had extrahepatic lesions, detected either by preoperative imaging or discovered at exploration. Two resections of apparently localized lesions were followed by rapid and aggressive recurrence. Five patients were treated 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 previously undergone a resection) died of tumor recurrence. One patient with advanced tumor died while waiting for transplantation. The remaining 4 patients are free of symptoms and have stable hepatic and extrahepatic disease. HEHE is nearly always multifocal, and our results with resection were dismal. Because of the unpredictable nature of the tumor, the indications for transplantation in patients without liver-related symptoms should be carefully evaluated. Nevertheless, extrahepatic disease should not be an absolute contraindication for liver transplantation in patients with severe liver dysfunction.

We report a case of de novo hepatocellular carcinoma (HCC) in a patient with recurrent hepatitis C (HCV) and cirrhosis 7 years after orthotopic liver transplantation (OLT). This is a previously unreported observation in the natural history of posttransplantantion HCV infection and reiterates the strong oncogenic potential of HCV. Copyright 1999 by the American Association for the Study of Liver DiseasesA 63-year-old white man underwent OLT on July 6, 1990, for end-stage liver disease caused by chronic non A non B hepatitis and alcohol abuse. Ultrasonography before transplantation did not show a focal lesion. Serum alphafetoprotein level was 10.1 mg/mL (normal, 20 mg/mL). The donor was a 65-year-old woman in whom the HCV status was not determined at the time of organ donation. The preperfusion and postperfusion liver biopsy specimens obtained around the time of transplantation showed lipofuscinosis, a finding consistent with the age of the donor, but there was no evidence of hepatitis. The explanted liver, sectioned every 0.5 cm, showed micronodular cirrhosis, moderate lymphocytic septal inflammation, mild piecemeal necrosis, and areas of multiacinar collapse. There was no malignancy. The patient was started on cyclosporine, 125 mg twice daily; azathioprine, 100 mg four times daily; and prednisone, 20 mg four times daily. Six months later, he had biopsy-proven hepatitis and positive HCV RNA in the serum detected by reverse-transcriptase polymerase chain reaction. Over the next 6 months, the hepatitis progressed to stage III, in transition to cirrhosis. The azathioprine dose was reduced to 75 mg four times daily 12 months after OLT and to 50 mg four times daily at 18 months. Two years after OLT, the prednisone dose was reduced to 5 mg four times daily. Three and one half years later, he was maintained on cyclosporine, 75 mg twice daily, and prednisone, 5 mg four times daily every other day, with no evidence of rejection. Blood cyclosporine levels were maintained at 150 to 200 mg/mL. He remained clinically stable for the next 6 years, when he underwent a second OLT for a decompensated liver. Ultrasonography before transplantation showed cirrhosis with a 1.4-cm solid hypoechoeic mass. Serum alphafetoprotein level was 73.6 mg/ mL.The explanted liver showed chronic hepatitis and cirrhosis, with a well-demarcated, hemorrhagic, and focally necrotic 1.5-cm nodule in the right lobe. Microscopically, this was a welldifferentiated HCC arising in a macroregenerative nodule. Fluorescent in situ hybridization for X and Y chromosomes showed the tumor to be XX, confirming its de novo origin in the engrafted liver. The patient died of sepsis 75 days after the surgery.

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