Department of Dermatology, Fundación Jiménez Díaz, Universidad Autonoma de Madrid, Madrid, Spain.
JAMA Dermatol. 2013 Apr;149(4):459-65. doi: 10.1001/jamadermatol.2013.3190.
Epithelioid sarcomalike (pseudomyogenic) hemangioendothelioma (ES-H) is a recently described and little-known vascular neoplasm that frequently presents with dermatologic lesions. Histopathologic characterization includes sheets or fascicles of plump, spindled and epithelioid, rhabdomyoblastlike neoplastic cells involving the dermis and often extending to subcutaneous tissue. Immunohistochemical analysis reveals neoplastic cells that show a constant immunophenotype characterized by immunoreactivity for cytokeratins and endothelial markers.
We described the clinical, histopathologic, and immunohistochemical features of 2 cases of cutaneous ES-H. Clinical examination revealed multifocal lesions that consisted of erythematous nodules on the leg and foot in case 1 and small perioral papules in case 2. Neoplastic cells had a rhabdomyoblastic appearance, with large nuclei and ample eosinophilic cytoplasm. Immunohistochemical analysis revealed expression of cytokeratin AE1/AE3, CD31, ERG, and FLI-1, with focal and weak positivity for CAM 5.2 and smooth muscle actin. The nuclei of neoplastic cells showed intact expression of INI-1. This immunoprofile, especially the ERG positivity, demonstrated the endothelial nature of proliferating cells.
We recommend adding the low-grade neoplasm ES-H to the large list of cutaneous vascular proliferations. Dermatologists should be aware of this low-grade cutaneous vascular tumor.
上皮样肉瘤样（假肌源性）血管内皮细胞瘤（ES-H）是一种最近描述的、鲜为人知的血管肿瘤，常伴有皮肤病变。组织病理学特征包括真皮和皮下组织常累及的片状或束状的肥胖、梭形和上皮样、横纹肌样肿瘤细胞。免疫组织化学分析显示肿瘤细胞具有恒定的免疫表型，表现为细胞角蛋白和内皮标记物的免疫反应性。
我们描述了 2 例皮肤 ES-H 的临床、组织病理学和免疫组织化学特征。临床检查显示，病例 1 下肢和足部有红斑结节，病例 2 有小的口周丘疹，这些病变均为多灶性病变。肿瘤细胞具有横纹肌样外观，细胞核大，胞质丰富嗜酸性。免疫组织化学分析显示细胞角蛋白 AE1/AE3、CD31、ERG 和 FLI-1 的表达，CAM5.2 和平滑肌肌动蛋白的表达呈局灶性和弱阳性。肿瘤细胞的核显示 INI-1 的完整表达。这种免疫表型，特别是 ERG 的阳性，表明增殖细胞的内皮性质。
我们建议将低度恶性肿瘤 ES-H 加入到大量的皮肤血管增生中。皮肤科医生应该意识到这种低度皮肤血管肿瘤。
