Background: Hepatic epithelioid hemangioendothelioma (HEHE) is a rare tumor and no standard treatment has been established. This study was aimed to retrospectively investigate the outcomes of different managements for HEHE patients
Methods: From March 2017 to November 2019, a retrospective investigation was performed among 50 HEHE 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: For all the 50 HEHE patients, 80% were asymptomatic and 94% had multiple intrahepatic lesions. Extrahepatic metastases were detected in 54% patients and 82% patients were radiologically misdiagnosed. For 18 patients with initial observation, 16 (88.9%) of them had progressive disease (PD). For 12 patients with curative intent surgery or radiofrequency ablation (RF), 10 (83.3%) of them had recurrence. Six patients with interferon-α had results of 4 partial response (PR), one complete response (CR) and one stable disease (SD). For 6 patients with thalidomide, 4 patients had PD and 2 patients had SD. Four patients with chemotherapy had 3 PD and one SD. Five patients with targeted therapy had 2 PR (both with apatinib), 2 PD and one SD.
Conclusions: HEHE patients had a high chance of PD during observation. The risk of recurrence after surgery or RF was high. Interferon-α and apatinib were the managements with tumor response (PR or CR). The encouraging result of interferon-α makes it a promising treatment for HEHE.