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Hepatic Epithelioid Hemangioendothelioma and Adult Liver Transplantation: Proposal for a Prognostic Score Based on the Analysis of the ELTR-ELITA Registry

Lai, Quirino MD, PhD; Feys, Estelle MD; Karam, Vincent PhD; Adam, Rene MD, PhD; Klempnauer, Jurgen MD; Oliverius, Martin MD, PhD; Mazzaferro, Vincenzo MD, PhD; Pascher, Andreas MD, PhD; Remiszewski, Piotr MD, PhD; Isoniemi, Helena MD, PhD; Pirenne, Jacques MD, PhD; Foss, Aksel MD, PhD; Ericzon, Bo G. MD, PhD; Markovic, Sasa MD, PhD; Lerut, Jan P. MD, PhDfor the European Liver Intestine Transplant Association (ELITA)

doi: 10.1097/TP.0000000000001603
Original Clinical Science—Liver

Background Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor which has an intermediate aggressive behavior. Although the value of liver transplantation (LT) is well established, its place in the management of HEHE is still unclear. The aim of this study is to confirm, based on a very large patient cohort, the value of LT in the management of HEHE and to identify risk factors for post-LT recurrence.

Methods The outcome of 149 transplant recipients with HEHE recorded in the European Liver Transplant Registry during the period November 1984 to May 2014 was analyzed. Median post-LT follow-up was 7.6 years (interquartile range, 2.8-14.4).

Results Cox regression analysis showed that macrovascular invasion (hazard ratio [HR], 4.8; P < 0.001), pre-LT waiting time of 120 days or less (HR, 2.6; P = 0.01) and hilar lymph node invasion (HR = 2.2; P = 0.03), but not pre-LT extrahepatic disease, were significant risk factors for recurrence. These findings, which were also confirmed in a propensity score analysis, allowed the development of a HEHE-LT score enabling stratification of patients in relation to their risk of tumor recurrence. Patients with a score of 2 or less had a much better 5-year disease-free survival compared to those having a score of 6 or higher (93.9% vs 38.5%; P < 0.001).

Conclusions The analysis of this (largest in the world) HEHE adult liver recipient cohort clearly confirms the value of LT in the treatment of this rare disorder and also permits identification of patients at risk of posttransplant recurrence. Posttransplant follow-up should take the HEHE-LT score into account. Extrahepatic disease localization is reconfirmed not to be a contraindication for LT.

This study updates the ELTR experience with HEHE. The findings from this larger cohort substantiate the role of OLT in HEHE and identify rapid transplantation and microvascular invasion as predictors of poor outcomes while extrahepatic disease is not. The authors also affirm the overall improvement in outcomes that seem primarily reflective of improved patient selection.

1 Starzl Abdominal Transplant Unit, University Hospitals St. Luc, Université catholique Louvain, Brussels, Belgium.

2 Centre Hépatobiliaire Paul Brousse, Paris, France.

3 Medizinsche Hochschule Hannover, Hannover, Germany.

4 Transplancenter IKEM, Prague, Czech Republic.

5 Istituto Nazionale Tumori, Milano, Italy.

6 Charité Campus Virchow Klinikum, Berlin, Germany.

7 Department of General Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland.

8 UCJ Helsingfors, Helsinki, Finland.

9 Department Abdominal transplantation Surgery, Universitaire Ziekenhuizen Gasthuisberg KULeuven, Belgium.

10 Rikshospitalet, Oslo Universitetssykehus, Oslo, Norway.

11 Karolinska University Hospital, Huddinge, Stockholm, Sweden.

12 University Medical Centre Ljubljana, Ljubljana, Slovenia.

Received 26 April 2016. Revision received 30 October 2016.

Accepted 7 November 2016.

The ELTR is supported in part by unrestricted grants from Astellas, Novartis and Roche and logistic support of the Paul Brousse Hospital-Assistance Publique, Hôpitaux de Paris (FR). The ELTR is a service of ELITA.

The order of the authors is determined following the guidelines for publication approved by the boards of European Liver and Intestinal Transplant Association (ELITA) and European Liver Transplant Registry (ELTR) and according to the number of transplantations recorded in the ELTR.

The authors declare no funding or conflicts of interest.

QL and JL were responsible for the conception, design and analysis of the study and for writing the final report; Q.L., E.F. and J.L. were involved with the collection and interpretation of data; V.K., R.A., J.K., M.O., V.M., A.P., P.R., H.I., J.P., A.F., B.G.E. and S.M. participated in data management and manuscript review.

Correspondence: Jan Lerut, MD, PhD, FACS, FEBTS, Starzl Abdominal Transplant Unit, University Hospitals Saint Luc, Université catholique Louvain (UCL), Avenue Hippocrates 10, 1200 Brussels, Belgium. (

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