肝脏单形性上皮样血管平滑肌脂肪瘤

目的：探讨肝脏单形性上皮样血管平滑肌脂肪瘤的临床病理学特征及诊断、鉴别诊断要点。方法：对1例单形性上皮样血管平滑肌脂肪瘤进行临床病理学分析及免疫组织化学研究。结果：肝脏单形性上皮样血管平滑肌脂肪瘤临床多无症状，光镜下单形性上皮样血管平滑肌脂肪瘤由形态多样的上皮样细胞构成，胞质透明或嗜酸，无脂肪组织及异常血管；免疫表型；HMB45阳性，SMA及vimentin部分阳性，desmin少数阳性，S－100蛋白弱阳性，cytokeratin及AFP阴性，CD34血管内皮细胞阳性。结论：肝脏单形性上皮样血管平滑肌脂肪瘤是极为罕见的间叶性肿瘤，组织起源至今不明，其诊断及鉴别诊断主要依靠病理组织学及免疫组织化学。

Hepatic monotypic epithelioid angiomyolipoma:A case report and literature review

Purpose To explore the clinicopathologic features and differential diagnosis of hepatic monotypic epithelioid angiomyolipoma(MEA). Methods One case of MEA was analyzed with light microscopy and immunohistochemistry. Results No clinical symptoms were found. The tumour was composed of pleomorphic epithelioid cells with clear and eosinophilic cytoplasm.Neither adipocytes nor abnormal vessels were recognized in the tumor.Immunohistochemically,the tumour cells were positive for HMB45,focally positive for smooth muscle actin(SMA),desmin and vimentin,weakly positive for S 100 protein and negative for cytokeratins and alpha fetoprotein(AFP);CD34 was positive for blood vessel endothelial cells. Conclusion MEA of the liver is a rare neoplasm.The pathogenesis is controversial.The diagnosis of MEA mainly relies on histopathological and immunohistochemical findings.