1. Hepatic Epithelioid Hemangioendothelioma and Adult Liver Transplantation
- Author
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Quirino, Lai, Estelle, Feys, Vincent, Karam, Rene, Adam, Jurgen, Klempnauer, Martin, Oliverius, Vincenzo, Mazzaferro, Andreas, Pascher, Piotr, Remiszewski, Helena, Isoniemi, Jacques, Pirenne, Aksel, Foss, Bo G, Ericzon, Sasa, Markovic, Jan P, Lerut, and Philippe, Wolf
- Subjects
Male ,Time Factors ,medicine.medical_treatment ,Kaplan-Meier Estimate ,Liver transplantation ,Gastroenterology ,Hemangioendothelioma ,0302 clinical medicine ,Risk Factors ,Registries ,Neoadjuvant therapy ,Graft Survival ,Liver Neoplasms ,Middle Aged ,Neoadjuvant Therapy ,Europe ,Treatment Outcome ,Lymphatic Metastasis ,030220 oncology & carcinogenesis ,Predictive value of tests ,Hemangioendothelioma, Epithelioid ,Female ,030211 gastroenterology & hepatology ,Algorithms ,medicine.drug ,Adult ,medicine.medical_specialty ,Waiting Lists ,Bevacizumab ,Risk Assessment ,Disease-Free Survival ,Decision Support Techniques ,03 medical and health sciences ,algorithms ,disease-free survival ,female ,graft survival ,hemangioendothelioma epithelioid ,humans ,Kaplan-Meier estimate ,liver neoplasms ,lymphatic metastasis ,male ,middle aged ,multivariate analysis ,neoadjuvant therapy ,neoplasm Invasiveness ,neoplasm recurrence local ,patient selection ,predictive value of tests ,propensity score ,proportional hazards models ,registries ,retrospective studies ,risk assessment ,risk factors ,time factors ,treatment outcome ,waiting lists ,decision support techniques ,liver transplantation ,transplantation ,Predictive Value of Tests ,Internal medicine ,medicine ,Humans ,Neoplasm Invasiveness ,Propensity Score ,Epithelioid hemangioendothelioma ,Proportional Hazards Models ,Retrospective Studies ,Transplantation ,business.industry ,Patient Selection ,Retrospective cohort study ,medicine.disease ,Liver Transplantation ,Surgery ,Multivariate Analysis ,Neoplasm Recurrence, Local ,business - Abstract
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.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).Cox regression analysis showed that macrovascular invasion (hazard ratio [HR], 4.8; P0.001), 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%; P0.001).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.
- Published
- 2017
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