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Prognosis of hepatic epithelioid hemangioendothelioma after living donor liver transplantation

대한이식학회지 2021년 35권 1호 p.15 ~ 23
나병곤, 황신, 안철수, 김기헌, 문덕복, 하태용, 송기원, 정동환, 박길춘, 윤영인, 강우형, 조휘동, 김상훈, 홍승모, 이승규,
소속 상세정보
나병곤 ( Na Byeong-Gon ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
황신 ( Hwang Shin ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
안철수 ( Ahn Chul-Soo ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
김기헌 ( Kim Ki-Hun ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
문덕복 ( Moon Deok-Bog ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
하태용 ( Ha Tae-Yong ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
송기원 ( Song Gi-Won ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
정동환 ( Jung Dong-Hwan ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
박길춘 ( Park Gil-Chun ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
윤영인 ( Yoon Young-In ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
강우형 ( Kang Woo-Hyoung ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
조휘동 ( Cho Hwui-Dong ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
김상훈 ( Kim Sang-Hoon ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery
홍승모 ( Hong Seung-Mo ) - University of Ulsan College of Medicine Asan Medical Center Department of Pathology
이승규 ( Lee Sung-Gyu ) - University of Ulsan College of Medicine Asan Medical Center Department of Surgery

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.

키워드

Borderline tumor; Recurrence; Vascular tumor; Metastasis; Immunohistochemical stain

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