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Hepatic Epithelioid Hemangioendothelioma as a Rare Indication for Liver Transplantation
Xiana Rial1, Javier López-Monclús1, José Luis Lucena1
1General and Digestive Surgery Department, Puerta de Hierro-Majadahonda University Hospital, Madrid, Spain.
Background:
Hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin, with nonspecific presentation and unpredictable clinical course. Several therapeutic options are available according to the literature, including chemotherapy and radiotherapy, liver resection (LR), and liver transplantation (LT).
Methods:
We present 2 cases of patients with HEHE treated with liver transplantation in our center, a 30-year-old man and a 42-year-old woman, diagnosed with several lesions involving both liver lobes.
Results:
Levels of tumor markers (carcinoembryonic antigen [CEA], cancer antigen 19.9 [Ca19.9], and alpha-fetoprotein [AFP]) were negative. Percutaneous biopsy revealed HEHE in both cases. Due to the bilobar location, liver transplantation was performed. Postoperative course was unremarkable, and no significant complications developed. At this time, 10 and 5 years' follow-up has concluded, respectively. Both patients remain alive, asymptomatic, with normal liver function and no sign of recurrence.
Conclusions:
HEHE is an uncommon malignant hepatic disease. Most cases present with bilobar involvement, and LT is probably the most useful treatment with favorable outcomes according to the literature and our experience. Further studies are needed to establish the optimal management of this rare entity.
