title: Epithelioid hemangioendothelioma of the liver as a rare indication for liver transplantation.
abstract: OBJECTIVE
  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 χ(2) 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 (χ(2) test = 1.784, df = 2, P = 0.409).
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Piotr
authors.initials: P
authors.lastname: Remiszewski
authors.affiliation: 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.
authors.forename: Ewa
authors.initials: E
authors.lastname: Szczerba
authors.affiliation: 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.
authors.forename: Piotr
authors.initials: P
authors.lastname: Kalinowski
authors.affiliation: 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.
authors.forename: Beata
authors.initials: B
authors.lastname: Gierej
authors.affiliation: 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.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Dudek
authors.affiliation: 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.
authors.forename: Mariusz
authors.initials: M
authors.lastname: Grodzicki
authors.affiliation: 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.
authors.forename: Marcin
authors.initials: M
authors.lastname: Kotulski
authors.affiliation: 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.
authors.forename: Rafał
authors.initials: R
authors.lastname: Paluszkiewicz
authors.affiliation: 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.
authors.forename: Waldemar
authors.initials: W
authors.lastname: Patkowski
authors.affiliation: 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.
authors.forename: Krzysztof
authors.initials: K
authors.lastname: Zieniewicz
authors.affiliation: 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.
authors.forename: Marek
authors.initials: M
authors.lastname: Krawczyk
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis; D016009:Chi-Square Distribution; D005260:Female; D018323:Hemangioendothelioma, Epithelioid / Q000737:chemistry / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007150:Immunohistochemistry; D053208:Kaplan-Meier Estimate; D008111:Liver Function Tests; D008113:Liver Neoplasms / Q000737:chemistry / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011044:Poland; D011237:Predictive Value of Tests; D012086:Reoperation; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor
keywords: Hemangioendothelioma; Liver malignancies; Liver transplantation; Transplantation indications; Transplantation results