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目的探讨子宫苗勒管腺肉瘤伴肉瘤过度生长(M櫣llerianadenosarcomaoftheuteruswithsarcomatousovergrowth,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M櫣llerian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M櫣llerian腺肉瘤相比,它更具有侵袭性,预后差。
Objective To investigate the clinicopathological features, biological behavior and prognosis of Müllerian adenosarcoma with endometrial disease and glomerular sarcoma (MASO). Methods 3 cases of MASO using light microscopy, immunohistochemical staining, and review the relevant literature. Results Three patients showed irregular vaginal bleeding, vaginal bleeding after menopause and uterine enlargement. In general, the intrauterine polypoid tumor, diameter 2 ~ 13cm, 2 cases of superficial myometrial invasion, deep infiltration of deep myometrial serosa. Histologically, three cases of MASO were seen typical adenosarcoma structure, which is composed of benign glands and low grade malignant sarcoma-like components, of which the proportion of pure sarcoma were more than 25%, and the sarcoma level than the adjacent adenosarcoma Example 2 contains heterologous components. 3 cases of sarcoma showed vimentin positive, while EMA, CD10, desmin, SMA, S-100 protein were negative. All patients underwent hysterectomy and bilateral accessory resection, and 1 patient had pelvic lymph node dissection. Two patients were followed up for 3 years and 5 years, respectively, without tumor-free survival, 1 patient died after 1 month. Conclusions MASO is a rare M 櫣 llerian mixed epithelial and stromal tumor with unique clinicopathological features. It is more aggressive and has a poorer prognosis than the classical uterine M 櫣 llerian adenosarcoma.