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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 investigate the clinicopathological and immunohistochemical features of rhabdomyosarcoma in children in Xinjiang. Methods Retrospective analysis of 18 cases of children with rhabdomyosarcoma in Xinjiang clinical and pathological data and immunological phenotype. Results There were 9 boys and 9 girls. The age of onset was from 10 months to 16 years old, with an average of 5.3 years. 88.9% of children were less than 10 years old. Among them, 5 are Han, 10 are Uygur and 3 are other nationalities. Primary in the head and neck in 8 cases, 4 limbs, genitourinary system in 2 cases, 2 cases of trunk, lung and pelvic in 1 case. Including embryonal rhabdomyosarcoma in 16 cases (including grape-like rhabdomyosarcoma in 1 case), alveolar rhabdomyosarcoma in 2 cases. Immunohistochemistry showed 18 cases of vimentin (+), 17 cases of desmin (+), 10 cases of CD99 (+), 11 cases of Myo D1 (+), 16 cases of myogenin +), While acinar rhabdomyosarcoma was diffuse (+). Conclusion Histological examination combined with immunohistochemistry is helpful for the diagnosis and differential diagnosis of rhabdomyosarcoma.