Treatment modalities and long-term outcomes of hepatic hemangioendothelioma in the United States

Background: We analyzed the outcomes of patients with hepatic epithelioid hemangioendothelioma (HEHE) in the United States after stratification by their most definitive treatment. Methods: The National Cancer Data Base was used to identify patients with HEHE between 2004 and 2018. Patients were divided in four treatment groups: no surgical therapy, ablation, liver resection or liver transplantation. Demographics and clinical characteristics were compared, and Kaplan Meier functions and Cox-regression were used for unadjusted and adjusted survival analyses. Results: Among a total of 334 patients, 218 (65.2%) were managed non-surgically, 74 (22.1%) underwent hepatic resections, 35 (10.4%) underwent liver transplantation and 7 (2.1%) underwent ablations. The overall median survival was 111 months (95%CI 73-149) after liver transplantation, 69 months (95%CI 45-92) after hepatic resection, 38 months (95%CI 0-78) after ablation and 80 months (95%CI 70-90) for patients managed by watchful waiting (P < 0.001). After adjustment, patients who underwent liver transplantation were found to have a better survival when compared to other therapies (Hazard Ratio: 0.61, 95% Confidence Interval: 0.38-0.97, p = 0.035). Conclusions: This study reports the outcomes of the largest cohort of patients with HEHE. The longest survival was observed after liver transplantation, followed by non-surgical management and hepatic resection. Because of selection bias, future studies to better characterize what criteria should be used for the selection of treatment modalities for HEHE are urgently needed.