子宫及阔韧带血管周上皮样细胞肿瘤6例临床病理分析

Perivascular epithelioid cell tumor of uterine and broad ligament: a clinicopathologic analysis

目的探讨子宫及阔韧带血管周上皮样细胞肿瘤的临床病理特征、生物学行为及诊断与鉴别诊断。方法收集6例子宫及阔韧带血管周上皮样细胞肿瘤的临床及病理资料,采用免疫组化EnVision法检测HMB45、SMA、S-100、CD-117和desmin。结果 6例患者平均年龄43.5岁,5例位于宫体,1例位于阔韧带。光镜下肿瘤细胞由透亮或嗜酸性的上皮样细胞构成,有些区域夹杂有分支样的血管,2例出现较多核分裂象与凝固性坏死。免疫组化显示6例HMB45均(+),SMA呈散在或弥漫(+)。2例术中见转移。6例患者随访5年均存活。结论子宫及阔韧带血管周上皮样细胞肿瘤的诊断依赖于形态学与免疫组化。其生物学行为需要进一步研究,但核分裂象与凝固性坏死可能是其恶性生物学行为的表现。

Objective To study the pathologic features, biological behavior, pathological diagnosis and differential diagnosis of uterine perivascular epitbelioid tumor （PEComa）. Methods Six cases of uterine and broad ligament perivaseular cell tumor were enrolled into the study. Immunohistochemical study for HMB-45, smooth muscle actin, S-100, CD-117, desmin etc. was carried out. Results The mean age of the patients was 43.5 years. Five tumors were located in the uterine corpus, and one presented in the broad ligament. All the six tumors were composed of clear or eosinophilie cells, associated with abundant small vessels in the stroma. Two cases had rather high mitotic rate and coagulative necrosis. Immunohistoehemically, the tumor cells demonstrated positive staining for HMB-45. The staining for desmin, CD117 and S-100 was negative. SMA positive cells were diffusely distributed in four cases and scattered in the other two cases. Two cases were diagnosed as malignant ones because of metastasis of tumor. All the patients were followed up for five years and they were still alive, with no evidence of recurrence. Conclusion The diagnosis of uterine and broad ligament PEComas is based on morphology and immunohistochemistry. Its biological behavior is needed to further study, but much mitosis and eoagulative necrosis may be associated with a poor prognosis.