子宫颈血管周上皮样细胞瘤临床病理观察

1 case report of perivascular epithelioid cell tumor of uterine cervix： cUnicopathological observation and literature review

目的探讨子宫颈血管周上皮样细胞瘤的临床病理特征、免疫组化及鉴别诊断。方法对1例子宫颈血管周上皮样细胞瘤进行组织形态学、免疫组化观察和文献复习。结果组织学上显示肿瘤主要由上皮样细胞围绕网状或裂隙样的薄壁小血管呈片状排列。免疫组化黑色素标记物、平滑肌肌动蛋白、波形蛋白、神经内分泌标记阳性。结论子宫颈血管周上皮样细胞瘤是一种由间充质发生的罕见的具有恶性潜能的肿瘤。这类少见的肿瘤在组织学及免疫组织化学上是以肌源性和黑色素标志物共同表达为主要特征。应与宫颈上皮样平滑肌肿瘤及黑色素肿瘤、子宫颈内膜间质肉瘤、透明细胞肉瘤等相鉴别。该病的预后尚无明确定论，需要从不断的报道中积累和总结经验。

Objective To explore histopathological and immunohistochemical characteristics and differential diagnosis of perivascular epithelioid cell tumor （PEComa） of the cervix. Methods Histomorphology and immunohistoehemistry of one case of PEComa of the cervix were observed and the related literatures were reviewed. Results Histologically, the tumour is mainly composed of epitheliod cells surrounding many little blood vessels with thin wall and arranging in forms of reticulation or cranny. Immunohisoehemieally, the tumour cells were positive for HMB45, SMA, Vimetin and neuroendoerine labels. Conclusion PEComa of the cervix is a rare potentially malignant tumor which arises from mesenchymal tissues. Main characteristic histological features and immunohistochemical profile of the tumor were co-expression of myogenic markers and melanogenesis-related markers. The diagnosis of the tumor should be differented from bizarre leiomyoma of the cervix, melanoma, endometrial stromal sarcoma, clear cell sarcoma and so on. The prognosis of this entity is undetermined due largely to the small number of reported cases.