肝脏恶性血管周上皮样细胞瘤临床病理观察

Malignant Neoplasm with Perivascular Epithelioid Cell Differentiation of the Liver:A Clinicopathologic Observation

目的探讨肝脏血管周上皮样细胞瘤的临床病理特点和诊断标准。方法对1例恶性病例的临床、影像学和病理特征进行观察并复习文献。结果患者因肿瘤复发死亡。肿物切面灰红灰黄色,质软易碎;镜下见梭形-上皮样瘤细胞围绕薄壁血管放射状排列,核异型深染,可见核分裂象;免疫组化示瘤细胞Vimentin、SMA和MelanA阳性表达,HMB45和CD117阴性表达,Ki-67指数10%。结论肝脏恶性血管周上皮样细胞瘤异型显著,可见核分裂象,CD117阴性表达,Ki-67指数高。

Objective To study the clinicopathologic features and diagnostic criteria of neoplasm with perivascular epithelioid cell differentiation （PEComa） of the liver. Methods A malignant case was analyzed with pathological morphology and immunohistochemical staining and review of the related literature. Results The woman was died of recurrence. On pathologic examination,the lesion appeared pale tan to yellow,soft,and friable. On light microscopy,the tumor showed spindle-epithelioid cells growing outward from the capillary vasculature. The nuclei were middle pleomorphism and mitosis was found. The neoplastic cells expressed vimentin,SMA and MelanA,but did not express HMB45 and CDll7. About 10% cells expressed Ki-67. Conclusion Malignant PEComa of the liver is characteristic of obvious cytologic atypia,high mitotic rate and Ki-67 index,but without CD117 expression.