Retrospective Study
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World J Gastroenterol. Aug 28, 2014; 20(32): 11333-11339
Published online Aug 28, 2014. doi: 10.3748/wjg.v20.i32.11333
Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation
Piotr Remiszewski, Ewa Szczerba, Piotr Kalinowski, Beata Gierej, Krzysztof Dudek, Mariusz Grodzicki, Marcin Kotulski, Rafał Paluszkiewicz, Waldemar Patkowski, Krzysztof Zieniewicz, Marek Krawczyk
Piotr Remiszewski, Ewa Szczerba, Piotr Kalinowski, Krzysztof Dudek, Mariusz Grodzicki, Marcin Kotulski, Rafał Paluszkiewicz, Waldemar Patkowski, Krzysztof Zieniewicz, Marek Krawczyk, Department of General, Transplant and Liver Surgery, Medical University of Warsaw, 00-097 Warsaw, Poland
Beata Gierej, Department of Anatomopathology, Medical University of Warsaw, 00-097 Warsaw, Poland
Author contributions: Remiszewski P, Szczerba E, Dudek K, Grodzicki M, Kotulski M, Paluszkiewicz R, Patkowski W, Zieniewicz K and Krawczyk M designed the research; Remiszewski P, Szczerba E and Gierej B analyzed the data; Remiszewski P, Szczerba E, Kalinowski P and Gierej B wrote the paper.
Correspondence to: Piotr Remiszewski, MD, PhD, Department of General, Transplant and Liver Surgery, Medical University of Warsaw, ul. Banacha 1a, 00-097 Warsaw, Poland. remi@mp.pl
Telephone: +48-22-5991545  Fax: +48-22-5992359
Received: December 27, 2013
Revised: February 25, 2014
Accepted: May 29, 2014
Published online: August 28, 2014
Abstract

AIM: To investigate the indications and outcomes of liver transplantation for hepatic epithelioid hemangioendothelioma (HEHE).

METHODS: Between 1989 and August 2013, in the Department of General, Transplant, and Liver Surgery, Medical University of Warsaw, 1306 orthotopic liver transplantations (OLTx) were performed, including 72 retransplantations. Unresectable HEHE was an indication for OLTx in 10 patients (0.8% of primary OLTx), the mean age of the patients was 40.5 ± 13.3 years (range 23-65 years), and the male-to-female ratio was 2:8. Kaplan-Meier survival analysis in HEHE, hepatocellular carcinoma (HCC), and other OLTx recipients groups was performed. The differences in mortality were compared using the χ2 test. A P-value < 0.05 indicated statistical significance.

RESULTS: No concomitant liver disease was found in any patient. There was no neoadjuvant chemotherapy or radiotherapy. Liver function test results were normal in most of the patients. The levels of alpha-fetoprotein, carcinoembryonic antigen, and carbohydrate antigen 19-9 were normal. In immunohistochemical staining, the neoplastic cells were positive for factor VIII-related antigen, CD31, and CD34, which are endothelial cell markers, and negative for cytokeratin 19, cytokeratin 7, and HepPar-1. Nine patients were alive without tumor recurrence. One patient died 2 mo after OLTx due to septic complications. No morbidity was observed. Maximum follow-up was 11.4 years, with a minimum of 1 mo. The cumulative survival rate at the end of follow-up in HEHE patients was 87.5% compared with 54.3% in the HCC group and 76.3% in the other OLTx recipients group (χ2 test = 1.784, df = 2, P = 0.409).

CONCLUSION: Unresectable HEHE, without extrahepatic metastases is an excellent indication for liver transplantation. Long-term survival is very good and much better than in HCC patients and the entire group of OLTx patients.

Keywords: Hemangioendothelioma, Liver transplantation, Liver malignancies, Transplantation results, Transplantation indications

Core tip: Epithelioid hemangioendothelioma (EHE) of the liver (hepatic EHE, HEHE) is a very rare tumor of mesenchymal origin. It typically occurs in female patients aged 20-40 years. HEHE is resistant to chemotherapy. Unresectable tumor, limited to the liver, may be a good indication for liver transplantation. The aim of this paper was to analyze the indications and outcomes of liver transplantation for HEHE.