Liver Transplantation for Hepatic Epithelioid Hemangioendothelioma - Retrospective Cohort

Introduction: Hepatic epithelioid hemangioendothelioma (HEH) is a rare neoplasm of unknown etiology and guarded prognosis if not diagnosed and treated properly. Resection is the primary treatment, but it is difficult to perform in large and multiple tumors, and liver transplantation (LT) is the most commonly used treatment in these cases. Objectives: To describe and analyze the clinical presentation, treatment, and prognosis of patients diagnosed with HEH and submitted to LT. Methods: Retrospective analysis of the clinical presentation, treatment, and prognosis of 8 patients undergoing LT due to HEH in two reference hospitals in the Northeast of Brazil. Results: A total of 2,413 liver transplants were carried out in two tertiary hospitals, of which only 8 (0.3%) were due to HEH. The median age at transplant was 36.7 (IQR [33.91–38.02]). The most frequent symptom was abdominal pain in the right hypochondrium, present in 6 patients (75.0%). One patient (12.5%) had pretransplant ascites. The calculated MELD was 10 ± 4.44, while the MELD corrected for special situations was 24.25 ± 3.41. One patient progressed to death due to recurrence of the disease with lung metastasis. The remaining patients (87.5%) are being followed up as outpatients with preserved liver function. The overall survival rate for patients at 1 month, 1 year, and 5 years was 100%, 87.5%, and 87.5%, respectively. Conclusion: HEH is a rare tumor of which the most common presentation is multinodular. This makes LT the most appropriate treatment with satisfactory long-term results.