肾脏及肾外横纹肌样瘤4例报告并文献分析

Renal and extra-renal rhabdoid tumor: analysis of 4 cases and literature review

目的:探讨横纹肌样瘤的临床病理特点、诊断及治疗。方法:收集天津医科大学肿瘤医院自2000年1月至2014年9月收治的4例横纹肌样瘤患者的病例资料,结合文献进行回顾性分析。结果:4例横纹肌样瘤患者中1例原发于肾脏、其余3例原发于肾外软组织。病理组织学上肿瘤细胞弥漫性生长,核仁突出,胞浆可见嗜酸性包涵体,核分裂多见。免疫组织化学显示波形蛋白(Vimentin)、上皮膜抗原(EMA)阳性,广谱角蛋白(CK)、CD99、CD34、S-100均有不同程度阳性,人肌调节蛋白1(Myo D1)、结合蛋白(Desmin)、整合酶相互作用分子-1(INI-1)阴性。结论:横纹肌样瘤是罕见且具有高度侵袭性的肿瘤,好发于肾脏,也可见于其他系统,其病理形态独特,需要结合免疫组织化学染色诊断。

Objective：To analyze the clinico-pathological characteristics, pathological diagnosis, and treatment of rhabdoid tu-mor. Methods：The medical records of four rhabdoid tumor patients that were admitted to the Tianjin Medical University Cancer Insti-tute and Hospital since 2000 were analyzed based on existing literature. Results：In one of the four cases, the tumor originated from the kidney, whereas in the other three, the tumor occurred from extra-renal soft tissues. Histologic analysis revealed that the tumor cells were loosely arranged with diffuse growth, vesicular nuclei, dyed cytoplasm, visible eosinophilic inclusions, and more nuclear fission. The results of immunohistochemical staining showed that the vimentin and epithelial membrane antigen were positive, whereas CK, CD99, CD34, and S-100 were positive at different degrees. MyoD1, Desmin, and INI-1 were negative. Conclusion：Rhabdoid tumor is rare and highly aggressive. It occurs mainly in the kidney and can also be found in other systems. The unique pathological form and im-munohistochemical staining observed on the tumor can be used as reference for diagnosis.