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目的:探讨腹膜后血管周上皮样细胞肿瘤(perivascular epithelioid cell neoplasms,PEComas)的临床和病理学特点,提高对PEComas的认识和诊断水平。方法:对1例腹膜后恶性PEComas进行临床病理分析及免疫组织化学研究,并复习相关文献。结果:肿瘤组织主要由上皮样细胞构成,胞质丰富、透明或嗜酸性颗粒状,间质富于血管。免疫组织化学显示:肿瘤细胞平滑肌肌动蛋白、HMB45,melan A及Desmin阳性,上皮膜抗原和vimentin局灶阳性,CgA,Syn,S-100,CD117,CD34,细胞角蛋白,calretinin及inhibin均阴性。结论:腹膜后PEComas是一种非常罕见的肿瘤,具有独特的组织学和免疫组织化学特征,应与腹膜后其他上皮样细胞肿瘤进行鉴别。
Objective: To investigate the clinical and pathological characteristics of perivascular epithelioid cell neoplasms (PEComas) and to improve their understanding and diagnosis of PEComas. Methods: One case of retroperitoneal malignant PEComas was studied by clinicopathological analysis and immunohistochemistry. The related literatures were reviewed. Results: The tumor tissue consisted mainly of epithelial cells, with rich cytoplasm, transparent or eosinophilic granulosa and interstitial vascularity. Immunohistochemistry showed that the positive expression of smooth muscle actin, HMB45, melan A and Desmin in tumor cells was positive in epithelial membrane antigen and vimentin, but negative in CgA, Syn, S-100, CD117, CD34, cytokeratin, calretinin and inhibin . CONCLUSIONS: Retroperitoneal PEComas are a rare type of tumor with unique histological and immunohistochemical features that should be differentiated from other retroperitoneal epithelioid tumor.