新疆地区儿童横纹肌肉瘤18例临床病理分析  

Childhood rhabdomyosarcoma: a clinicopathological study of 18 cases in Xinjiang

作　　者：宋新兰[1] 付娟娟[1] 

机构地区：[1]新疆维吾尔自治区人民医院病理科,乌鲁木齐830001

出　　处：《诊断病理学杂志》2015年第8期475-478,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨新疆地区儿童横纹肌肉瘤的临床病理及免疫组化特点。方法回顾性分析新疆地区18例儿童横纹肌肉瘤的临床病理资料及免疫学表型。结果男童9例,女童9例,发病年龄10个月-16岁,平均5.3岁;88.9%的患儿发病〈10岁。其中汉族5例,维吾尔族10例,其他民族3例。原发于头颈部8例,四肢4例,泌尿生殖系统2例,躯干2例,肺部及盆腔各1例。其中胚胎性横纹肌肉瘤16例（其中葡萄状横纹肌肉瘤1例）,腺泡状横纹肌肉瘤2例。免疫组化示18例vimentin均（＋）,17例desmin（＋）,10例CD99（＋）,11例Myo D1（＋）;16例myogenin（＋）,其中胚胎性横纹肌肉瘤myogenin呈部分（＋）,而腺泡状横纹肌肉瘤呈弥漫（＋）。结论组织学形态结合免疫组化检查有利于横纹肌肉瘤的诊断与鉴别诊断。Objective To study the clinicopathological, immunohistochemical characteristics of pediatric rhabdomyosarcomas（ RMS） in Xinjiang. Methods We retrospectively reviewed the clinical data and immunological phenotype in 18 cases of childhood rhabdomyosarcoma. Results There were 9 male and 9 female patients with ages ranging from 10 months to 16 years with a mean of 5. 3 years; 88. 9% of them occured in children before the age of 10 years. There were 5 cases of Han nationality and 10 cases of Uyghur nationality,and 3 cases of other ethnic nationality.The primary sites were found in head and neck（ 8 cases）,extremities（ 4 cases）,genitourinary system（ 2 cases）,trunk（ 2 case）,lung（ 1 case） and pelvis（ 1 case）. Histological types included embryonal rhabdomyosarcoma（ 16 cases）,in which there was 1 case of botryoid rhabdomyosarcoma and two cases of alveolar rhabdomyosarcoma.Immunohistochemically,the positive rates for vimentin,desmin,CD99,Myo D1 and myogenin were 100%,94. 4%,55. 6%,61. 1%,and 88. 9%,respectively. Myogenin was partially expressed in embryonal rhabdomyosarcoma,and more diffusely expressed in alveolar rhabdomyosarcoma. Conclusions Histological and immunohistochemistry are helpful in diagnosis and differential diagnosis of RMS.

关 键 词：软组织肿瘤 横纹肌肉瘤 免疫组化 

分 类 号：R738.7[医药卫生—肿瘤]