子宫苗勒腺肉瘤6例临床病理学分析并文献复习

Uterus Müllerian adenosarcoma:a clinicopathologic analysis of 6 cases and review of literature

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。

Purpose To observe the clinicopathologic and immunophenotyping features in 6 cases of uterus Müllerian adenosarcoma（UMA）,in order to increase the diagnosis,differential diagnosis and therapy levels.Methods Six cases were reviewed with clinical data analysis,morphological assessment,immunophenotyping and follow-up.Results Four cases occurred in uterine endometrium and 2 cases occurred in cervix.The main clinical manifestations were abnormal vaginal bleeding and pelvic pain.Physical examination showed enlarged uterus and a pelvic mass.All patients received surgical treatment and 4 had postoperative chemotherapy.2 cases received radiotherapy.4 cases of uterine endometrial low-grade adenosarcoma received postoperative chemotherapy with no relapse so far;2 cervical adenosarcomas with heterogenous chondrosarcoma differentiation relapsed and died after 2 years postoperation.All cases were seen cystic dilation,crack-like glands and stroma concentrated around gland forming periglandular cuffs.Stroma mitotic figures were 2~12/10 HPF.One case presented with chondrosarcoma component accompanied by a high grade pleomorphic sarcoma component overgrowth and myometrial invasion.All the cases expressed CK,4 cases of endometrial lesions expressed ER,PR,vimentin but negative for CD10 and CK in stroma cells.1 case expressed SMA and desmin,3 cases expressed CD10,2 cases with chondrosarcoma differentiation expressed S-100 protein.Ki-67 proliferation index was 1% in epithelial component and 4% in stroma cells of low grade adenosarcoma,but 20% in high-grade sarcoma associated sarcomatous overgrowth.Conclusion UMA is a rare tumor without specific clinical symptoms and signs.The diagnosis depends on morphologic examination.The tumors show low malignant potential and the vast majority are at early stage.Surgical excision is the main treatment with a good prognosis in the early stage disease with complete removal of tumors.The prognosis is poor in advanced adenosarcoma with sarcomatous overgrowth.Due to the relatively high rate of recurrence,long-term follow-up is recommended.