女性生殖道苗勒腺肉瘤9例临床病理学分析

Müllerian adenosarcoma of the female genital tract: a clinicopathologic analysis of 9 cases

目的分析9例女性生殖道苗勒腺肉瘤(female genital tract Müllerian adenosarcoma,FGTMA)的临床病理特征、免疫表型,提高对该病的认识。方法回顾性分析病理确诊的9例FGTMA的临床和组织学特征及免疫表型,并随访观察。结果临床主要表现为阴道不规则流血、盆腔包块和子宫颈肿物。全部行手术治疗,5例术后辅以化疗,1例放疗。肿瘤由良性腺上皮和肉瘤性间质组成。5例伴有异源性成分,其中4例同时伴肉瘤样过度生长。免疫表型:腺上皮细胞角蛋白(CK)均阳性;间质波形蛋白(vimentin)均阳性,2例P53均≥75%细胞弥漫阳性,5例P16阳性,MutS同源蛋白2(MSH2)、MutS同源蛋白6(MSH6)、Mut L同源蛋白1(MLH1)1例同时阴性。Ki-67增殖指数5%~45%。结论 FGTMA临床无特异性,确诊依赖病检。早期且完整切除病灶者预后较好,晚期且合并肉瘤样过度生长者预后差。

Objective To investigate the clinicopathologic features and immunophenotypes of female genital tract Müllerian adenosarcoma(FGTMA) in 9 cases and to improve understanding of the disease. Methods A retrospective study was conducted on the clinicopathologic features and immunophenotypes of FGTMA in 9 cases. All the patients received postoperative follow-up. Results The majoR clinical symptoms of FGTMA were irregulaRvaginal bleeding, pelvic masses and cervical lesions. All the patients received surgical treatment, among whom 5 cases received adjuvant postoperative chemotherapy and 1 case received radiotherapy. Histopathologically, the tumors were composed of benign glandulaRepitheliums and malignant mesenchymal tissues. The sarcomatousstroma contained heterologous elements in 5 cases, among whom 4 cases had concomitant sarcomatous overgrowth. Immunohistochemically, glandulaR epitheliums showed expression of cytokeratin(CK) and mesenchymal tissues showed vimentin expression in all the cases, among whom 2 cases showed strong expression of P53 (more than 75% cells with scattered positive expression), and 5 cases showed P16 expression, and 1 case was negative to MutS homolog 2(MSH2), MutS homolog 6(MSH6) and Mut L homologue 1(MLH1). The proliferation index of Ki-67 was 5%~45%. Conclusion There are no specific clinical features of FGTMA. Accurate diagnosis of the disease relies on pathological examination. Patients receiving early diagnosis and complete surgical treatment have betteR outcomes than those with advance disease and sarcomatous overgrowth.