Liu Xiaolei, Zhou Ruiquan, Si Shuang, Liu Liguo, Yang Shiwei, Han Dongdong, Tan Haidong
Second Department of Hepatopancreatobiliary Surgery, China-Japan Friendship Hospital, Beijing, China.
Front Oncol. 2022 Aug 24;12:972306. doi: 10.3389/fonc.2022.972306. eCollection 2022.
Hepatic epithelioid hemangioendothelioma (HEH) is a very rare tumor originated from vascular endothelial cells, with unpredictable malignancy. No standard treatment has been established yet. Although surgical resection and liver transplantation have been reported to be effective treatments with favorable long-term outcomes, the multiple intrahepatic lesions or extrahepatic metastasis makes these procedures unsuitable to most patients. Sirolimus was reported to be an effective drug for epithelioid hemangioendothelioma but only about 10% achieved partial response. Interferon-alpha 2b (IFN-a 2b) has also been used for the treatment of HEH, and the rate of tumor regression was more than 50%. Here, we report a HEH patient with giant intrahepatic tumor (>15cm), who achieved partial response after the combined therapy of sirolimus and IFN-a 2b. The giant intrahepatic lesion (>15 cm) regressed obviously after 8 months treatment and no severe adverse event was reported. The good response and safety of combined therapy with sirolimus and IFN-a 2b provide a promising guidance for future clinical study.
肝上皮样血管内皮瘤（HEH）是一种非常罕见的起源于血管内皮细胞的肿瘤，其恶性程度难以预测。目前尚未确立标准治疗方法。尽管手术切除和肝移植已被报道为有效的治疗方法且长期预后良好，但肝内多发病灶或肝外转移使这些方法不适用于大多数患者。西罗莫司据报道是治疗上皮样血管内皮瘤的有效药物，但只有约10%的患者获得部分缓解。干扰素-α 2b（IFN-α 2b）也已用于HEH的治疗，肿瘤消退率超过50%。在此，我们报告一例患有巨大肝内肿瘤（>15cm）的HEH患者，其在西罗莫司和IFN-α 2b联合治疗后获得部分缓解。经过8个月的治疗，巨大肝内病灶（>15cm）明显缩小，且未报告严重不良事件。西罗莫司和IFN-α 2b联合治疗的良好疗效和安全性为未来的临床研究提供了有前景的指导。
