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目的观察6例子宫苗勒腺肉瘤(uterus Müllerian adenosarcoma,UMA)的临床病理特点、免疫表型,提高对其诊断、鉴别诊断和治疗水平。方法回顾性分析病理确诊的6例UMA临床特点、肿瘤组织形态学特征及肿瘤免疫表型,并随访观察。结果 6例患者4例发生于子宫内膜,2例发生于宫颈。临床主要表现为阴道异常出血、盆腔疼痛和子宫颈息肉。全部行手术治疗,4例术后辅以化疗,2例放疗。4例发生于子宫内膜者形态学均为低级别腺肉瘤,术后辅以化疗至今无复发;2例发生于宫颈者均合并有软骨肉瘤异源性成分,术后辅助放、化疗2年后因肿瘤复发死亡。镜下所有病例均可见不规则囊性扩张、裂隙样腺体和肿瘤间质以腺周套袖样及息肉样突入腺腔的形态学特征,肿瘤间质核分裂象2～12个/10 HPF。2例伴有软骨肉瘤异源性成分者,其中1例同时伴有高级别多形性肉瘤成分过度生长并浸润肌层。免疫表型:6例肿瘤上皮CK均呈强阳性表达;上皮:ER、PR和vimentin均(+),CD10(-),Ki-67增殖指数1%;肉瘤间质:全部表达vimentin,4例表达ER、PR;1例表达SMA、desmin,3例表达CD10,Ki-67增殖指数4%;2例伴有软骨肉瘤分化者局灶表达S-100蛋白。间质细胞Ki-67增殖指数20%,明显高于低级别腺肉瘤4%的表达。结论 UMA临床罕见,患者症状、体征无特异性,正确诊断依赖病理形态学观察。低级别腺肉瘤治疗应采用手术为主的综合疗法,早期且完整切除病灶者预后较好,晚期且合并肉瘤成分过度生长者及伴有异源性分化者预后差。肿瘤的复发率较高,应长期密切随访。
Objective To observe the clinicopathological features, immunophenotypes of 6 uterine leptomeninosarcomas (UMA) and to improve their diagnosis, differential diagnosis and treatment. Methods Retrospective analysis of 6 cases of pathologically confirmed UMA clinical features, tumor histomorphology and tumor immunophenotype, and follow-up observation. Results 6 patients occurred in 4 cases of endometrial, 2 cases occurred in the cervix. The main clinical manifestations of abnormal vaginal bleeding, pelvic pain and cervical polyps. All patients underwent surgery, 4 patients received adjuvant chemotherapy and 2 patients received radiotherapy. 4 cases of endometrial morphology were low grade adenosarcoma, postoperative adjuvant chemotherapy has not relapsed; 2 cases occurred in the cervix were combined with chondrosarcoma heterologous components, postoperative adjuvant radiotherapy and chemotherapy for 2 years After death due to tumor recurrence. All cases under microscope showed irregular cystic dilatation, glandular fissure and tumor interstitial glandular sleeve and polypoid into the glandular cavity morphological characteristics of the tumor stromal mitosis 2 ~ 12/10 HPF. Two patients with chondrosarcoma heterologous components, including one case accompanied by high-grade pleomorphic sarcoma ingredients over-growth and infiltration of muscle. Immunophenotype: 6 cases of tumor epithelial CK were strongly positive expression; epithelial: ER, PR and vimentin were (+), CD10 (-), Ki-67 proliferation index 1%; sarcoma interstitial: all expressed vimentin, 4 cases One case expressed SMA, desmin, three cases expressed CD10, the proliferation index of Ki-67 was 4%. Two cases were associated with the expression of S-100 protein in chondrosarcoma differentiation. Interstitial cell Ki-67 proliferation index 20%, significantly higher than 4% of low-grade adenosarcoma expression. Conclusion UMA clinical rare, symptoms and signs of patients without specificity, correct diagnosis depends on pathological observation. The treatment of low-grade adenosarcoma surgery should be based on the use of comprehensive treatment, early and complete removal of the lesion better prognosis, advanced sarcoma and overgrowth of those with heterologous differentiation poor prognosis. Tumor recurrence rate is higher, should be long-term close follow-up.