子宫Müllerian腺肉瘤伴间质横纹肌样瘤分化1例并文献复习

Adenosarcoma of the uterus with mesenchymal rhabdoid tumor differentiation:a case report and review of literature

目的探讨子宫Müllerian腺肉瘤伴间质横纹肌样瘤分化的临床病理特征。方法用光镜、组织化学及免疫组化方法观察其病理组织学表现。结果肿瘤由良性上皮成分和肉瘤性间质成分组成,肉瘤成分过度生长。肿瘤细胞弥漫浸润性分布,细胞大,胞质丰富嗜酸性并可见嗜伊红包涵体。免疫表型:vim entin、CK、NF、CD57、CD99、CgA、Syn阳性,SMA散在阳性,而desm in、EMA、CD10、GFAP、MyoD1、Inh ib in-α、HMB45和S-100蛋白阴性。组织化学染色PAS阴性,网状纤维染色显示网状纤维包绕单个或小巢肉瘤性间质细胞。结论子宫腺肉瘤伴间质横纹肌样瘤分化是一种罕见的混合性Müllerian肿瘤,应与子宫内膜间质肉瘤、子宫横纹肌肉瘤和低分化癌等鉴别。

Purpose To study the clinical and pathological features of Muellerian adenosarcoma of the uterue with mesenchymal rhabdoid tumor differentiation. Methods Light microscopy, histochemistry and immunohistochemistry methods were utilized to study the feature of the histopathology of Muellerian adenosarcoma of the uterue with mesenchymal rhabdoid tumor differentiation. Results The tumor was composed with a benign glandular and a malignant stromal component, with sarcomatous overgrowth. The tumor cells showed diffuse and infiltrative characteristic, with rich and eosinophilic cytoplasm , in some of which eosinophilic globular bodies were seen. Immunohistochemistry showed reactivity for vimentin, cytokeratin, neurofilament, CD57, CD99, chromogranin A and synaptophysin, sparse reactivity for SMA, but negative for desmin, EMA, CD10, GFAP, MyoD1, Inhibin-α, HMB45 and S-100 protein. No expression of PAS, reticular fiber surrounding single or several tumor cells can be showed by Ag staining. Conclusions Muellerian adenosarcoma of the uterue with mesenchymal rhabdoid tumor differentiation is a rare type of mixed Muellerian tumor , and the differential disgnosis of this tumor includes Endometrial stromal scarcoma, Rhabdomyosarcoma of the uterus and poorly differentiated carcinoma.