Xiaolei Liu,* Peijun Yang,* Liguo Liu, Shuang Si, Ruiquan Zhou, Tiantong Liu, Haidong Tan

Second Department of Hepatopancreatobiliary Surgery, China-Japan Friendship Hospital, Beijing, People’s Republic of China

*These authors contributed equally to this work

Correspondence: Xiaolei Liu; Haidong Tan, Second Department of Hepatopancreatobiliary Surgery, China-Japan Friendship Hospital, 2 Yinghua Dongjie, Hepingli, Beijing, 100029, People’s Republic of China, Email liuxiaolei0302@126.com; hpblt_cjfh@126.com

Objective: To evaluate the clinical efficacy of nab-paclitaxel based chemotherapy in the treatment of advanced epithelioid hemangioendothelioma (EHE).
Methods: Since March 2022, chemotherapy has been recommended for patients with advanced EHE characterized by large tumors (liver tumors > 10 cm or tumors in other organs > 3 cm), rapid tumor progression, severe symptoms, serosal effusion, and treatment failure. Two chemotherapy regimens were administered: nab-paclitaxel plus bevacizumab and nab-paclitaxel plus sirolimus. Clinical data and outcomes were retrospectively analyzed.
Results: From March 2022 to August 2024, 21 patients with histologically confirmed EHE who received nab-paclitaxel based chemotherapy were included. At baseline, 18 patients (85.7%) presented with tumor-related symptoms, and serosal effusion was detected in 12 patients (57.1%). Among patients with hepatic EHE, six (28.6%) had tumors > 10 cm, while six (28.6%) with EHE at other sites had tumors > 3 cm. Partial response and stable disease were achieved in 5 (23.8%) and 12 (57.1%) patients, respectively, resulting in a disease control rate of 80.9%. Symptom relief was observed in 15 of 18 patients (83.3%), and decreased serosal effusion was noted in 6 of 12 patients (50.0%). The 1- and 2-year progression-free survival rates were 50.7% and 13.5%, respectively, while the 1- and 2-year overall survival rates were 70.6% and 51.5%, respectively.
Conclusion: Nab-paclitaxel based chemotherapy may offer an effective treatment option for patients with advanced EHE exhibiting adverse prognostic factors. However, further clinical trials are required to confirm its efficacy.

Keywords: epithelioid hemangioendothelioma, EHE, chemotherapy, sarcoma, survival