Yang You-ping, Zhu Yang-li, Wu Wei-ping, Wang Zhao-ming, Zhang Jian-min
Department of Pathology, Zhejiang Wenling Second People's Hospital, Wenling 317500, China.
Zhonghua Bing Li Xue Za Zhi. 2007 May;36(5):302-6.
To study the pathologic features, diagnosis, differential diagnosis and biologic behavior of uterine perivascular epithelioid tumor.
Five cases of uterine perivascular epithelioid cell tumor were studied by light microscopy and immunohistochemistry. Follow-up information was reviewed.
All the five tumors were composed by clear or eosinophilic cells arranged in nests and cords, associated with abundant small vessels and hyalinization in the stroma. Immunohistochemically, the tumor cells demonstrated positive staining for melanocytic markers (HMB45 and/or Melan-A), desmin and smooth muscle actin. The staining for cytokeratin and CD10 was negative. All the patients followed for a certain period are still alive, with no evidence of disease recurrence.
Perivascular epithelioid cell tumor is a rare mesenchymal tumor of uterus, with distinctive histologic and immunohistochemical features. It should be distinguished from clear cell carcinoma and epithelioid leiomyoma of uterus. Positivity for melanocytic markers (especially HMB45) plays an important role in the diagnosis of this tumor. In general, the tumor is categorized as benign, with uncertain malignant potential and malignant.
研究子宫血管周上皮样细胞瘤的病理特征、诊断、鉴别诊断及生物学行为。
对5例子宫血管周上皮样细胞瘤进行光镜及免疫组化研究，并复习随访资料。
5例肿瘤均由透明或嗜酸性细胞组成，呈巢状和条索状排列，间质内有丰富的小血管及玻璃样变。免疫组化显示肿瘤细胞黑素细胞标记物（HMB45和/或Melan-A）、结蛋白和平滑肌肌动蛋白呈阳性染色，细胞角蛋白和CD10染色阴性。所有随访一定时间的患者均存活，无疾病复发迹象。
血管周上皮样细胞瘤是一种罕见的子宫间叶性肿瘤，具有独特的组织学和免疫组化特征。应与子宫透明细胞癌和上皮样平滑肌瘤相鉴别。黑素细胞标记物（尤其是HMB45）阳性在该肿瘤的诊断中起重要作用。总体而言，该肿瘤分为良性、恶性潜能不确定和恶性。
