血管周上皮样细胞肿瘤的临床病理学观察

Clinicopathological observation of neoplasms with perivascular epithelioid cell differention

目的探讨肾脏和肝脏血管平滑肌脂肪瘤(AML)、肺脏透明细胞"糖"瘤(CCST)、子宫血管周上皮样细胞肿瘤(PEComa)的共同病理学特征及共同的诊断和鉴别诊断要点。方法对肾脏和肝脏AML、肺脏CCST、子宫PEComa各1例的标本进行病理形态学和免疫组织化学观察。结果 4例不同部位和3种命名的肿瘤有相同的形态学,主要表现瘤细胞以上皮样细胞的组成,呈薄壁血管周围排列,免疫组化4例均HMB45阳性(4/4),3例SMA阳性(3/4)。结论上述发生在不同部位、不同命名的肿瘤,在形态和免疫组化上有共同特点:即血管周排列、瘤细胞呈上皮样;免疫组化表达黑色素标记物,亦可表达平滑肌标记物,这些特点支持统一为血管周上皮样细胞肿瘤(PEComas)的看法。

Objective To investigate the common pathological features,diagnosis,and differential diagnosis of angiomyolipoma（AML） of the kidney and liver,clear cell ＂sugar＂ tumor（CCST） of the lung,and perivascular epithelioid cell tumor（PEComa） of the uterus.Methods Each case of AML of the kidney and liver,CCST of the lung,and PEComa of the uterus was analyzed by light microscopy and immunohistochemistry.Results Four cases tumors of three different terms had the same morphology,mainly presented epithelioid neoplastic cell around thin-walled blood vessels.Immunohistochemically,HMB45 was positive in four cases（4/4）,and SMA was positive in three of four cases（3/4）.Conclusion Though the above mentioned tumors occur in different sites and have different terms.,they share common morphological and immunophenotypic features including epithelioid neoplastic cell around thin-walled blood vessels,positive melanocytic marker and smooth muscle marker.Our results support the view that these tumors belong to a larger family descriptively termed PEComas.