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成人胎儿型横纹肌瘤临床病理观察 被引量:1

Clinicopathologic features of fetal rhabdomyoma
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摘要 目的探讨胎儿型横纹肌瘤(FRM)的临床病理学特征、发病机制、诊断及鉴别诊断。方法对1例发生于成人右肩部的FRM进行临床病理分析及免疫组化研究,并复习相关文献。结果镜下见束带状、短梭形及卵圆形的肿瘤细胞交错分布,胞质丰富呈嗜伊红色,无细胞异型性、病理性核分裂象及坏死,有的瘤细胞可见横纹结构,瘤细胞间质局部呈黏液样。免疫组化:肿瘤细胞actin(HHF-35)、desmin、α-sarcomeric和vimentin均弥漫强(+),α-SMA散在(+),Ki-67增殖指数<5%;MyoD1、EMA、CD163和S-100均(-)。结论 FRM是一种罕见的良性横纹肌肿瘤,发生于成年男性者更为罕见。组织学上该肿瘤主要与高分化胚胎性横纹肌肉瘤相鉴别,后者往往具有显著的细胞异型性及病理性核分裂象。通常对FRM完整手术切除不会出现复发及转移。 Abstract: Objective To explore the clinicopathological characteristics of fetal rhabdomyoma (FRM) and to investigate its pathogenesis, diagnosis and differential diagnosis from other tumor. Methods Clinieopathologieal examination and immunohistochemical findings were analyzed in one case of FRM and the related literature was reviewed. Results Microscopically, the tumor was mainly composed of haphazardly arranged short, fascicular bundles of small to medium-sized spindle and oval cells. Most of the tumor ceils were large and had abundant eosinophilic cytoplasm. No evidence of cytological atypia, necrosis and increased mitotic activity were seen. Myxoid areas were sparse and crude striations were seen in some areas. Immunohistoehemically, tumor cells showed the strong and diffuse positivity for actin( HHF-35 ), desmin, ct-sareomeric and vimentin, sparsely for ct-SMA. The labeling index of Ki-67 was less than 5%. Stains were negative for MyoD1, EMA, CD163 and S-100. Conclusions FBM is an unusual benign soft tissue tumor arising from striated muscle. It occurs in adult male more rarely. The main considerations in the histologic differential diagnosis are embryonal rhabdomyosarcoma, which presents with marked nuclear atypia and atypical mitoses. Recurrence and metastasis after complete excision is extremely rare.
出处 《诊断病理学杂志》 CSCD 北大核心 2013年第1期36-38,共3页 Chinese Journal of Diagnostic Pathology
关键词 胎儿型横纹肌瘤 临床病理学 诊断 Fetal rhabdomyoma Clinicopathology Diagnosis
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参考文献10

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