ALK1阳性的实体型腺泡状横纹肌肉瘤1例并文献复习

发布时间：2018-04-25 19:38

本文选题：横纹肌肉瘤 + 实体型　；参考：《临床与实验病理学杂志》2017年11期

【摘要】：目的探讨ALK1阳性实体型腺泡状横纹肌肉瘤(solid alveolar rhabdomyosarcoma,SARMS)的临床病理学特征、诊断和鉴别诊断。方法采用HE染色进行形态学观察,应用免疫组化En Vision两步法、荧光原位杂交技术检测SARMS,并复习相关文献。结果 ALK1阳性SARMS瘤细胞呈实性腺泡状、片巢状结构分布,瘤细胞胞界清晰,胞质丰富,或嗜酸、或透明,核居中,圆形、多边形或梭形,核仁不明显,核分裂象和凋亡易见。免疫表型:vimentin、Myogenin、desmin、ALK1均阳性,Ki-67增殖指数约90%,上皮性标记、淋巴瘤标记及神经源性标记、恶性黑色素瘤标记、软组织肉瘤标记均阴性,网状纤维染色示腺泡状结构,ALK1荧光原位杂交结果示ALK1基因扩增,FOXO1(FKHR)基因分离。结论 SARMS是发生于中老年人少见的软组织恶性肿瘤,需结合其组织学特征、免疫组化及荧光原位杂交进行鉴别诊断。ALK1是横纹肌肉瘤组织学分型、治疗和判断预后的重要因子。
[Abstract]:Objective to investigate the clinicopathological features, diagnosis and differential diagnosis of solid alveolar rhabdomyosarcoma.com (ALK1) positive solid rhabdomyosarcoma. Methods HE staining was used to observe the morphology, and immunohistochemical en Vision two-step method was used to detect SARMS by fluorescence in situ hybridization, and the related literatures were reviewed. Results ALK1 positive SARMS tumor cells showed solid glandular vesicles, nesting structure, clear cytoplasm, acidophilic or transparent, central nucleus, round, polygonal or fusiform, not obvious nucleoli, mitosis and apoptosis. Immunophenotype: Myogenin desminminator ALK1 positive proliferative index was about 90%, epithelial labeling, lymphoma labeling and neurogenic labeling, malignant melanoma labeling, soft tissue sarcoma labeling negative. The results of fluorescence in situ hybridization of ALK1 showed that ALK1 gene was amplified by FXO1 FKHRA gene. Conclusion SARMS is a rare soft tissue malignant tumor in middle aged and old people. It is necessary to combine its histological features. Immunohistochemistry and fluorescence in situ hybridization are important factors in histologic classification, treatment and prognosis of rhabdomyosarcoma.
【作者单位】：山西医科大学基础医学院病理学教研室;山西省肿瘤医院病理科;山西省肿瘤医院结直肠外科;
【基金】：山西省自然科学基金(201601D011129)
【分类号】：R738.7

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