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目的探讨小儿恶性横纹肌样瘤(MRT)的临床病理特点、免疫表型、诊断与鉴别诊断。方法复习33例MRT的临床资料、组织切片,进行光镜检查和免疫组化染色。结果 33例MRT大部分为婴幼儿,肿瘤较大。其中肾14例,软组织11例,中枢神经系统8例。组织学特征是横纹肌样细胞和包涵体。免疫组化示CK、EMA和vimentin(+),INI1(-)。结论组织学上呈横纹肌样细胞,免疫组化CK、EMA和vimentin(+),INI1(-)是MRT诊断与鉴别诊断的关键。肿瘤高度恶性,需手术切除后辅以强力的化疗和放疗。
Objective To investigate the clinicopathological characteristics, immunophenotype, diagnosis and differential diagnosis of malignant rhabdomyosarcoma (MRT) in children. Methods The clinical data of 33 cases of MRT were reviewed. The sections were stained with light microscopy and immunohistochemistry. Results Most of the 33 cases of MRT were infants and young children with large tumors. Among them, 14 cases were kidney, 11 cases were soft tissue and 8 cases were central nervous system. Histological features are striated muscle-like cells and inclusion bodies. Immunohistochemistry showed CK, EMA and vimentin (+), INI1 (-). Conclusions Histological examination of striated cells showed that immunohistochemistry of CK, EMA and vimentin (+) and INI1 (-) are the key points in the diagnosis and differential diagnosis of MRT. The tumor is highly malignant and needs to be treated with powerful chemotherapy and radiotherapy after surgery.