儿童泌尿生殖系统横纹肌肉瘤临床病理分析

摘要

Objective To investigate pediatric genitourinary rhabdomyosarcoma ( RMS) ' s clinicopathologic features , immunophenotype , pathogenesis , imaging features , diagnosis and differential diagnosis , treatment and prognosis .Methods Review of 3 cases of pediatric genitourinary RMS patients admitted to the Second Affiliated Hospital of Kunming Medical Uni -versity, analyze the clinicopathological features , immunophenotype and review of the literature .Results Three cases of pedi-atric genitourinary RMS were male, age 3-14 years old;location:near the testis in 1 cases, 2 cases of bladder; histology:spindle cell RMS2 cases, RMS1 cases of botryoid;immunophenotype:3 cases of RMS were different degrees of expression of Desmin, MyoD1, Myoglobin, a few cases the expression of Myogenin , Ki67 index was 30%-60%underwent surgical resec-tion, radiotherapy and chemotherapy were not performed .Conclusion RMS is a rare soft tissue tumor in children , with a high degree of malignancy and poor prognosis .%目的 探讨儿童泌尿生殖系统横纹肌肉瘤(RMS)的临床病理学特征、免疫表型、发病机制、影像学特点、诊断和鉴别诊断、治疗及预后.方法 回顾昆明医科大学第二附属医院收治的3例儿童泌尿生殖系统RMS患者,分析其临床病理学特点、免疫表型并复习相关文献.结果 3例儿童泌尿生殖系统RMS均为男性,年龄3~14岁;发生部位:睾丸旁1例,膀胱2例;组织学:梭形细胞RMS 2例,葡萄簇状RMS 1例;免疫表型:3例RMS均不同程度的表达Desmin、MyoD1、Myoglobin,少部分表达Myogenin,Ki67指数为30%~60%均行手术切除,未进行放疗及化疗.结论 儿童泌尿生殖系统RMS是一种少见软组织肿瘤,恶性程度高,预后差.