子宫血管周上皮样细胞肿瘤临床病理观察

Perivascular epithelioid cell tumor of uterus:report of 5 cases and literature review

目的研究子宫血管周上皮样细胞肿瘤的病理学特征、诊断、鉴别诊断和生物学行为。方法对5例子宫血管周上皮样细胞肿瘤进行常规组织学和免疫组织化学(SP 法)染色和观察,对患者进行随访,并复习相关文献。结果光镜下5例肿瘤均由透明或嗜酸细胞巢或宽窄不等的细胞索组成,间质有丰富的小血管和程度不等的透明变。免疫组织化学染色示5例瘤细胞均黑色素细胞标记阳性和程度不等的结蛋白和平滑肌肌动蛋白(SMA)阳性,CK 和 CD10阴性。5例患者现均存活。结论子宫血管周上皮样细胞肿瘤具有较特征性的组织病理及免疫组织化学特点,HMB45阳性对诊断有重要作用。该肿瘤分良生、恶性潜能不能确定和恶性三类,应与透明细胞癌和上皮样平滑肌肿瘤区别。

Objective To study the pathologic features, diagnosis, differential diagnosis and biologic behavior of uterine perivascular epithelioid tumor. Methods Five cases of uterine perivascular epithelioid cell tumor were studied by light microscopy and immunohistochemistry. Follow-up information was reviewed. Results 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. Conclusions 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. Positlvity 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.