pISSN 2671-8790 eISSN 2671-8804

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Korean J Transplant 2021; 35(1): 15-23

Published online March 31, 2021

https://doi.org/10.4285/kjt.20.0049

© The Korean Society for Transplantation

Prognosis of hepatic epithelioid hemangioendothelioma after living donor liver transplantation

Byeong-Gon Na1 , Shin Hwang1 , Chul-Soo Ahn1 , Ki-Hun Kim1 , Deok-Bog Moon1 , Tae-Yong Ha1 , Gi-Won Song1 , Dong-Hwan Jung1 , Gil-Chun Park1 , Young-In Yoon1 , Woo-Hyoung Kang1 , Hwui-Dong Cho1 , Sang Hoon Kim1 , Seung-Mo Hong2 , Sung-Gyu Lee1

1Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
2Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea

Correspondence to: Shin Hwang
Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, 88 Olympic-ro 43-gil, Songpagu, Seoul 05505, Korea
Tel: +82-2-3010-3930
Fax: +82-2-3010-6701
E-mail: shwang@amc.seoul.kr

Received: October 12, 2020; Revised: November 5, 2020; Accepted: November 6, 2020

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Background: Epithelioid hemangioendothelioma (EHE) is a rare borderline vascular tumor. Due to its rarity and protean behavior, the optimal treatment of hepatic EHE has not yet been standardized. This single-center study describes outcomes in patients with hepatic EHE who underwent living donor liver transplantation (LDLT).
Methods: The medical records of patients who underwent LDLT for hepatic EHE from 2007 to 2016 were reviewed.
Results: During 10-year period, four patients, one man and three women, of mean age 41.3±11.1 years, underwent LDLT for hepatic EHE. Based on imaging modalities, these patients were preoperatively diagnosed with EHE or hepatocellular carcinoma, with percutaneous liver biopsy confirming that all four had hepatic EHE. The tumors were multiple and scattered over entire liver, precluding liver resection. Blood tumor markers were not elevated, except that CA19-9 and des-γ-carboxy prothrombin was slightly elevated in one patient. Mean model for end-stage liver disease score was 10.8±5.7. All patients underwent LDLT using modified right liver grafts, with graft-recipient weight ratio of 1.11±0.19, and all recovered uneventfully after LDLT. One patient died due to tumor recurrence at 9 months, whereas the other three have done well without tumor recurrence, resulting in 5-year disease-free and overall patient survival rates of 75% each. The patient with tumor recurrence was classified as a high-risk patient based on the original and modified hepatic EHE-LT scoring systems.
Conclusions: LDLT can be an effective treatment for patients with unresectable hepatic EHEs that are confined within the liver and absence of macrovascular invasion and lymph node metastasis.

Keywords: Borderline tumor, Recurrence, Vascular tumor, Metastasis, Immunohistochemical stain

HIGHLIGHTS
  • Four cases of hepatic epithelioid hemangioendothelioma who underwent living donor liver transplantation are presented in this study.

  • Living donor liver transplantation can be an effective treatment for patients with unresectable hepatic epithelioid hemangioendotheliomas that are confined within the liver and absence of macrovascular invasion and lymph node metastasis.