子宫外子宫内膜间质肉瘤1例病理分析及文献复习

目的:探讨子宫外子宫内膜间质肉瘤(endometrial stromal sarcoma, ESS)的组织形态学及免疫组织化学特点. 方法:应用光镜、透射电镜和免疫组织化学标记对1例子宫外原发性ESS进行观察并文献复习. 结果:本例子宫外原发性ESS病变为多发性,累及右卵巢、阑尾、结肠和大网膜等处,伴有右卵巢、阴道、阑尾、结肠等多脏器子宫内膜异位症. 瘤细胞由浸润性、弥漫性增生的单一性圆形或梭形细胞组成,小血管丰富,2~5个核分裂像/10HPF. 电镜观察示ESS瘤细胞超微结构近似正常子宫内膜间质细胞,具有纤维母细胞、平滑肌细胞的形态特征. 免疫组织化学标记示肿瘤组织vimentin,desmin,EMA,ER,PR阳性,而CK, SMA,S-100,LCA,CD34均为阴性. 结论:子宫外原发性ESS组织发生来自子宫内膜间质细胞,可能与子宫内膜异位症和激素的影响有密切关系. ESS具有向平滑肌、纤维母细胞和上皮细胞分化的特点.

A case report of extrauterine low-grade endometrial stromal sarcoma and review of literatures

AIM: To report a case of primary extrauterine endometrial stromal sarcoma (ESS) and discuss the morphologic and immunohistochemical features with a review of recent literatures. METHODS: The clinical characteristics, histological features and immunological properties of ESS were analyzed retrospectively using routine HE staining, transmission electron microscopic (TEM) and immunohistochemical methods. RESULTS: The reported case of primary extrauterine ESS in this article presented lesions in multiple organs and multiple foci, involving right ovary, appendix, colon and greater omentum. Islands of endometrial tissue with well formed glands and stroma were found in the case. Histologically, the primary extrauterine ESS was characterized by an infiltrative and diffuse proliferation of uniform round or oval cells, abundant small vessels, low mitotic activity, the presence of foam cells and vascular invasion. TEM revealed that the tumor cells resembled normal proliferative phase endometrial stromal cells and they showed variable degrees of smooth muscle differentiation and fibroblast differentiation. Immunohistochemically, the tumor cells were diffusely stained for vimentin, desmin, EMA, estrogen receptor (ER) and progesterone receptor (PR), but not stained for CK,SMA, S 100, LCA and CD34. CONCLUSION: These results indicate that primary extrauterine ESS originates from endometrial stromal cells, which may derive from the Mullerian system. The case demonstrates that extrauterine ESS may stem from a preexisting endometriosis and may have a potential for smooth muscle, fibroblast and epithelial differentiation. PR and ER are identified as positive in the case, which indicates hormonal sensitivity.