Case Report Chemoembolization followed by orthotopic liver transplant for epithelioid hemangioendothelioma Hepaticepithelioidhemangioendothelioma(HEHE) is a rare, low-grade neoplasm of endothelial origin. The first documentation of this unusual tumor emerged in the literature only 20 yr ago (1, 2). Because of an insidious nature and propensity for multicentric growth, 5-yr survival reported in the first large series was a discouraging 28% (2). Intro- duction of transplantation as a treatment option broughtnewhopeforcontrollingHEHE(3).Inthis report, we describe the first reported case of multi- centric, bilobar HEHE treated successfully with chemoembolization followed by transplantation. Case report Initial presentation A 46-yr-old man presented with new fullness of the left supraclavicular region. Computed tomography (CT) of the chest found mild extrathoracic adenopathy, and no intrathoracic pathology. How- ever, inferior images of the chest CT picked up ominous appearing hepatic lesions. Abdominal CT was the performed defining multiple lesions in the liver, bilobar, the largest of which was 6 cm in diameter and located just posterior to the portal vein in the right lobe (Fig. 1). The dominant lesion was biopsied under guidance. Histologic diagnosis of adenocarcinoma with unknown primary was established. In addition, biopsy revealed low- grade, chronic active hepatitis C without fibrosis. Specimens were sent to Armed Forces Institute of Pathology in Washington DC and determined to be HEHE via immunohistochemical staining. Management After evaluation by a general surgeon and medical oncologist, it was felt that metastatic supraclavic- ular disease prohibited definitive treatment. St Peter SD, Moss AA, Huettl EA, Leslie KO, Mulligan DC. Chemoembolization followed by orthotopic liver transplant for epithelioid hemangioendothelioma. Clin Transplant 2003: 17: 549–553. ª Blackwell Munksgaard, 2003 Abstract: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare liver tumor with an indolent course relative to other hepatic malignancies. Over the past two decades, primary treatment for these lesions has been defined as resection for localized disease, or transplantation for diffuse and multifocal tumors. No published report to date has described effective pre- or post-operative adjuvant treatment for this disease. In this report, we present the first case of HEHE effectively managed with chemoembolization followed by transplantation, documenting objective tumor response to embolization. Furthermore, diagnosis for this lesion can easily be mistaken, directing management in erroneous directions. This case illustrates diagnostic pitfalls affiliated with the work-up of this tumor. Shawn D St Peter a , Adyr A Moss a , Eric A Huettl b , Kevin O Leslie c and David C Mulligan a a Division of Transplant Surgery, b Division of Vascular and Interventional Radiology and c Department of Pathology, Mayo Clinic Scottsdale, Scottsdale, AZ, USA Key words: chemoembolization – epithelioid hemangioendothelioma – liver transplantation Corresponding author: David C. Mulligan MD, Chair, Divisions of Transplant, Hepatobiliary and Pancreatic Surgery, Mayo Clinic Hospital, 5E Transplant Dept, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA. Tel.: 602-342-0514; fax: 602-342-2324; e-mail: mulligan.david@mayo.edu Accepted for publication 13 December 2002 Clin Transplant 2003: 17: 549–553 Copyright ª Blackwell Munksgaard 2003 549