目的 探讨儿童肾恶性横纹肌样瘤的临床及病理特征、治疗方法及预后。 方法

目的 探讨儿童肾恶性横纹肌样瘤的临床及病理特征、治疗方法及预后。 方法 回顾分析我院2006年1月-2019年10月收治的4例肾恶性横纹肌样瘤患者的临床资料，及对手术标本进行组织切片，进行光镜检查及免疫组化染色。结果 4例患者，其中男2例、女2例，年龄4月-1岁8月，平均年龄10月，临床表现为腹部肿物及血尿，病理组织形态特征是具有大核仁、丰富胞质的横纹肌样细胞和经典嗜酸性胞质的包涵体。免疫组化示肿瘤细胞胞浆强表达Vimentin，灶状表达CK、EMA、CD99、CD34、WT1、S100、CyclinD1，不表达INI1、Desmin和Myogenin。结论 儿童肾恶性横纹肌样瘤是一种具有高度侵袭性的恶性肿瘤，易转移、易复发、预后差，治疗方式以手术根治肾切除术加化疗为主。

目的 探讨儿童肾恶性横纹肌样瘤的临床及病理特征、治疗方法及预后。 方法 回顾分析我院2006年1月-2019年10月收治的4例肾恶性横纹肌样瘤患者的临床资料，及对手术标本进行组织切片，进行光镜检查及免疫组化染色。 结果 4例患者，其中男2例、女2例，年龄4月-1岁8月，平均年龄10月，临床表现为腹部肿物及血尿，病理组织形态特征是具有大核仁、丰富胞质的横纹肌样细胞和经典嗜酸性胞质的包涵体。 免疫组化示肿瘤细胞胞浆强表达Vimentin，灶状表达CK、EMA、CD99、CD34、WT1、S100、CyclinD1，不表达INI1、Desmin和Myogenin。 结论 儿童肾恶性横纹肌样瘤是一种具有高度侵袭性的恶性肿瘤，易转移、易复发、预后差，治疗方式以手术根治肾切除术加化疗为主。

Objective to investigate the clinical and pathological features, treatment and prognosis of malignant rhabdomyoma of the kidney in children. Methods the clinical data of 4 cases of renal malignant rhabdomyoma from January 2006 to October 2019 in our hospital were analyzed retrospectively, and the tissue sections, light microscopy and immunohistochemical staining were performed on the surgical specimens. Results 4 patients, 2 male and 2 female, aged from April to August, with an average age of 10 months, presented with abdominal mass and hematuria. The histopathological features were rhabdomyoid cells with large nucleoli, abundant cytoplasm and inclusion bodies of classic eosinophils. Immunohistochemistry showed that vimentin was strongly expressed in the cytoplasm of tumor cells, CK, EMA, CD99, CD34, WT1, S100, CyclinD1 were expressed in focus, and INI1, desmin and myogenin were not expressed. Conclusion children's malignant rhabdomyoma is a kind of highly invasive malignant tumor, which is easy to metastasize, easy to recur and has poor prognosis.<br>