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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。
Objective To investigate the clinicopathological features, pathogenesis, diagnosis and differential diagnosis of fetal rhabdomyoma (FRM). Methods One case of FRM occurred in the right shoulder of adult was analyzed by clinicopathological and immunohistochemistry, and reviewed the related literatures. Results Microscopically, the bundles of short, fusiform and oval tumor cells were staggered. The cytoplasm was enriched with eosinophilic, acellular, pathological mitosis and necrosis. Some of the tumor cells showed striated structure, The tumor cell stroma is mucoid locally. The expression of MyoD1, EMA, CD163 and MyoD1 were significantly higher than those in the control group (P <0.05). Immunohistochemistry: The tumor cells actin (HHF-35), desmin, S-100 both (-). Conclusion FRM is a rare benign rhabdomyosarcoma that occurs more frequently in adult males. Histologically, the tumor is mainly differentiated from well-differentiated embryonal rhabdomyosarcomas, which often have significant cellular atypia and pathological mitosis. Frequently, there is no recurrence and metastasis of a complete surgical resection of the FRM.