HEPATIC EPITHELIOID HEMANGIO-ENDOTHELIOMA AND ADULT LIVER TRANSPLANTATION: PROPOSAL FOR A PROGNOSTIC SCORE BASED ON THE ANALYSIS OF THE ELTR-ELITA REGISTRY.

BACKGROUND: Hepatic epithelioid hemangio-endothelioma (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.

MATERIAL AND METHODS: the outcome of 149 transplant recipients with HEHE recorded in the European Liver Transplant Registry during the period November1984-May 2014 was analyzed. Median post-LT follow-up was 7.6 years (IQR=2.8-14.4).

RESULTS: Cox regression analysis showed that macro-vascular invasion (hazard ratio=4.8; p<0.001), pre-LT waiting-time ≤120 days (HR=2.6; p=0.01) and hilar lymph-node invasion (HR=2.2; p=0.03), but not pre-LT extra-hepatic disease (EHD), 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 had a much better 5-year disease-free survival compared to those having a score of ≥6 (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 be take the HEHE-LT score into account. EHD localization is re-confirmed not to be a contraindication for LT.