子宫苗勒氏腺肉瘤临床病理特征分析

Clinicopathologic analysis of uterus mullerian adenosarcoma

目的 探讨子宫苗勒氏腺肉瘤（uterine müllerian adenosarcoma,UMA）的临床病理特点,提高对UMA的诊断、鉴别诊断和治疗水平.方法 分析4例UMA的临床特点,肿瘤组织学形态特征及肿瘤免疫表型,并随访观察诊治结果及转归.结果 4例患者中3例发生于宫内膜,1例发生于宫颈.其中1例形态学为高级别腺肉瘤伴有横纹肌肉瘤异源性分化,并有肉瘤过度生长,手术后辅助化疗,2年半后肿瘤复发导致患者死亡;余3例中2例为低级别,1例为高级别,但术后均未进行放、化疗等抗肿瘤治疗,至今无复发.镜下所有病例均可见不规则囊性扩张或裂隙样腺体,肉瘤性间质以腺周袖套样（生发层）围绕腺体以及息肉样突入腺腔的形态学特征,肉瘤性间质核分裂像2～13个/10HPF.免疫表型：4例腺肉瘤上皮成分：CK、EMA均（＋）,其中宫内膜型上皮ER、PR、Vimentin均（＋）,CD10（-）、P53（-）,Ki-67增殖指数低于5％.肉瘤性间质全部强阳性表达Vimentin,而ER、PR、SMA、Desmin、P53表达不一,Ki-67增殖指数3％～20％.结论 低级别腺肉瘤行子宫切除术后复发率较低,高级别腺肉瘤伴肉瘤过度生长者及伴有异源分化者复发快,预后较差。

Objective To analyze the clinicopathologic features of uterine mullerian adenosarcoma （UMA）in order to improve the diagnosis,differential diagnosis and therapy levels. Methods The clinical features, histological morphology and immunophenotyping of 4 patients with uterine mullerian adenosarcoma were retrospectively analyzed. Results 3 cases occurred in uterine endometrium and 1 cases occurred in cervix. 1 cases was uterine endometrial high -level adeno- sarcoma with heterogenous Rhabdomyosarcoma differentiation. Two and a half years after adjuvant chemotherapy after surgery, the patient died by recurrence. 3 cases were uterine endometrial low-grade adenosarcoma. The patients did not receive postoperative radiotherapy or chemotherapy and did no relapse so far. All cases were seen irregular cystic dilatation or crack-like glands and sarcoma of stromal Cuff-like glandular week （germinal layer） around the glands and glandu- lar polypoidhroke morphological characteristics. The sarcomatous component mitotic figures were 2- 13/10 HPF. 4cases of epithelial component of adenosarcoma were positive for CK and EMA. Endometrial epithelial were positive for ER,PR, vimenti, but negative for CD10 and p53. Ki-67 proliferation index of less than 5%. The sarcomatous component were strong positive for Vimentin, while the expression of ER, PR, SMA, Desmin, P53 were varies. Ki-67 proliferation index was 3-20%. Conclusion Low level adenosarcoma hysterectomy has a lower recurrence rate. The high level adenosarcoma with sarcoma and accompanied by an overgrowth of heterologous differentiation has a fast recurrence and poor prognosis.