Objective: Hepatic epithelioid hemangioendothelioma (HEH) is an extremely rare tumor, and no standard treatment has been established yet. This study aimed to retrospectively investigate the outcomes of different managements for HEH patients.
Patients and methods: From March 2014 to November 2019, a retrospective investigation was performed among 50 HEH patients to summarize the outcomes of different managements. Their medical records were collected, and the outcome of each management was evaluated based on radiological images.
Results: Of the 50 HEH patients examined, 80% were asymptomatic, and 94% had multiple intrahepatic lesions. Extrahepatic metastases were detected in 54% of patients, and 82% of patients were radiologically misdiagnosed. 16 (88.9%) of 18 patients with initial observation had progressive disease (PD). Of 12 patients with curatively intended surgery or radiofrequency ablation (RF), 10 (83.3%) of them had a recurrence. Six treated patients with interferon-α had results of 4 partial responses (PR), 1 complete response (CR), and 1 stable disease (SD). Of 6 patients with thalidomide, 4 patients had PD, and 2 patients had SD. Four patients treated with chemotherapy had 3 PD and 1 SD. Five patients treated with targeted therapy had 2 PR, 2 PD, and 1 SD.
Conclusions: During our observation, we found that HEH patients had a high chance of PD. The risk of recurrence after surgery or RF was high. The encouraging result of interferon-α therapy makes it a promising treatment for HEH.