上皮样血管内皮瘤（EHE）是一种起源于血管内皮细胞、低度恶性肿瘤，其特征为血管内皮细胞增殖。2002年，世界卫生组织将EHE分类为具有潜在转移能力的局部侵袭性肿瘤。目前，EHE的诊断基于病理学、组织学和免疫组织化学检查。目前没有标准的治疗指南。我们在此报道一位69岁男性，因左侧胸痛和腹痛超过2个月。另一家医院的胸腹部增强计算机断层扫描显示左侧肾上腺区有肿块，被认为是恶性的。我们医院的正电子发射计算机断层扫描显示左侧肾上腺区有一个大的多房、高代谢、囊性肿块，被认为是恶性的。因此，对肿块进行穿刺活检，并通过病理学检查（包括免疫组织化学染色）确认了EHE的诊断。该患者接受了采用PD-1免疫检查点抑制剂托瑞帕利单抗的治疗，并获得了长期的成功。最佳反应为稳定疾病（SD），无进展生存期（PFS）超过13个月。该患者至今仍然健在。由于先前研究的样本量较小，需要进一步研究以确定托瑞帕利单抗在EHE治疗方面的安全性和疗效。Copyright © 2023 Bu, Peng, Liu, He, Wang, Li, Niu and Zhang.

Epithelioid hemangioendotheliomas (EHEs), low-grade malignant tumors of vascular endothelial cell origin, are characterized by vascular endothelial proliferation. In 2002, the World Health Organization classified EHEs as locally aggressive tumors with the potential to metastasize. Currently, the diagnosis of EHE is based on pathology, histological and immunohistochemical examinations. There are no standard treatment guidelines. We here report a 69-year-old man who presented with left-sided chest and abdominal pain for more than 2 months. Enhanced computed tomography of the thorax and abdomen in another hospital suggested a mass in the left adrenal region that was considered malignant. Positron emission tomography- computed tomography in our hospital suggested a large multi-loculated, hypermetabolic, cystic mass in the left adrenal region that was considered malignant. Accordingly, a puncture biopsy of the mass was performed and the diagnosis of EHE confirmed by pathological examination, including immunohistochemical staining. This patient was treated with the programmed death 1 (PD-1) immune checkpoint inhibitor toripalimab with long-term success. The best response was stable disease (SD) with a progression-free survival (PFS) of more than 13 months. The patient is still alive now. Because the sample size of previous studies was small, further studies are needed to determine the safety and efficacy of toripalimab in the treatment of EHE.Copyright © 2023 Bu, Peng, Liu, He, Wang, Li, Niu and Zhang.