子宫Müllerian腺肉瘤伴肉瘤过度生长临床病理分析

Müllerian adenosarcoma of uterus with sarcomatous overgrowth:a report of 3 cases

目的 探讨子宫苗勒管腺肉瘤伴肉瘤过度生长（Müllerian adenosarcoma of the uterus with sarcomatous overgrowth，MASO）的临床病理特征、生物学行为及预后。方法 对3例MASO采用光镜、免疫组化染色观察，并复习相关文献。结果 3例患者分别表现为不规则阴道出血、绝经后阴道出血及子宫增大。大体上，宫腔内见息肉状肿物，直径2～13cm，2例浸润浅肌层，1例浸润深肌层达浆膜。组织学上，3例MASO均见典型腺肉瘤结构，即由良性腺体和低度恶性肉瘤样成分混合而成，其中纯粹肉瘤成分比例均超过25％，并且肉瘤级别比相邻的腺肉瘤高；例2含有异源成分。3例肉瘤成分均显示vimentin阳性，而EMA、CD10、desmin、SMA、S-100蛋白等均阴性。3例均行全子宫＋双侧附件切除术，1例进行了盆腔淋巴结清扫。2例术后分别随访3年及5年均无瘤生存，1例术后1个月死亡。结论 MASO是一种少见的Müllerian混合上皮和间质肿瘤，具有独特的临床病理形态特征；与经典的子宫Müllerian腺肉瘤相比，它更具有侵袭性，预后差。

Purpose To investigate the clinicopathological features of Müllerian adenosarcoma of the uterus with sarcomatous overgrowth （MASO） and its behavior and prognosis. Methods Three cases of MASO in the uterus were investigated by light microscopy and immunohistochemistry. The related literature was reviewed. Results The patients presented with abnormal or postmenopausal vaginal bleeding and an enlarged uterus, respectively. Cross examination of the uterus revealed polypoid masses filling the endometrial cavity that measured 2 to 13 cm in diameter. These masses invaded the subjacent myometrium in 2 cases, and extended to the serosal surface in 1 case. Histologically, each case was composed of a typical mullerian adenosarcoma （MA） characterized by an admixture of a benign glandular component and a sarcomatous, usually low-grade stromal component. The distinctive feature of the adenosarcoma was overgrowth by pure sarcoma that accounted for more than 25% of the tumor and when this sarcoma was higher grade than that of the associated adenosarcoma in three cases. One case had heterologous mesenchymal component. The sarcomatous component showed a positive reaction for vimentin, but was negative for EMA, CD10, desmin, SMA, S-100 protein in three cases. Three patients underwent the total abdominal hysterectomy and bilateral salpingo-oophorectomy, and pelvic lymph node dissection was performed in 1 case. On followup, 2 cases were alive with no evidence of disease at postoperative intervals of 3 and 5 years. One case died of disease 1 month after hysterectomy. Conclusions MASO of the uterus is an uncommon variant of mullerian mixed epithelial and mesenchymal tumor with distinctive clinical and pathological features. MASO of the uterus seems to be more aggressive than classic MA of the uterus associated with poor prognosis.