Wang Yibei, Niu Yiru, Mi Zhiyu, Zhao Jianhui, Zhang Ruoqi, Liu Jianfeng
Department of Otolaryngology-Head and Neck Surgery, China-Japan Friendship Hospital, Beijing 100029, P.R. China.
Department of Pathology, China-Japan Friendship Hospital, Beijing 100029, P.R. China.
Oncol Lett. 2024 Oct 21;29(1):17. doi: 10.3892/ol.2024.14763. eCollection 2025 Jan.
Epithelioid hemangioendothelioma (EHE) is a rare soft-tissue vascular neoplasm with a prevalence of one in one million. The present study firstly reports two cases of EHE occurring in the auricle. The clinical, histopathological and immunohistochemical features of two patients with auricular EHE are described, and the associated literature are reviewed. Two adult male patients each presented with an asymptomatic, unilateral soft skin-colored noninflammatory swelling of the auricle. Based on their clinical manifestations, both patients were initially diagnosed with auricular pseudocysts. Auricular excision surgery was performed under general anesthesia. The resected specimens were sent for pathological examination. Immunohistochemical examination showed that the specimens were positive for CD31, CD34, friend leukemia integration 1 (FLI-1), coagulation factor 8 and E26 transformation-specific-related gene, which was consistent with EHE. Follow-up after surgery showed no evidence of tumor recurrence. It may be concluded EHEs of low malignancy should be included in the differential diagnosis of patients with auricular pseudocysts. EHEs can be diagnosed based on their morphological and histological characteristics, with immunohistochemical positivity for FLI-1 and CD31 being suggestive of a diagnosis of EHE.
上皮样血管内皮瘤（EHE）是一种罕见的软组织血管肿瘤，发病率为百万分之一。本研究首次报告了两例发生于耳廓的EHE。描述了两名耳廓EHE患者的临床、组织病理学和免疫组化特征，并复习了相关文献。两名成年男性患者均表现为耳廓无症状、单侧、皮肤颜色正常的非炎性肿胀。根据临床表现，两名患者最初均被诊断为耳廓假性囊肿。在全身麻醉下进行了耳廓切除术。切除的标本送病理检查。免疫组化检查显示标本CD31、CD34、Friend白血病整合1（FLI-1）、凝血因子8和E26转化特异性相关基因呈阳性，符合EHE。术后随访未发现肿瘤复发迹象。可以得出结论，低恶性度的EHE应纳入耳廓假性囊肿患者的鉴别诊断。EHE可根据其形态和组织学特征进行诊断，FLI-1和CD31免疫组化阳性提示EHE诊断。
