儿童肾恶性横纹肌样瘤的临床及病理分析

Clinical and pathological analysis of malignant rhabdoid tumor of kidney in children

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。方法回顾性分析昆明市儿童医院2006年1月至2019年10月收治的4例肾恶性横纹肌样瘤患者的临床资料,对手术标本进行组织切片、光镜检查及免疫组织化学染色。结果 4例患者中男2例,女2例;年龄4个月至1岁8个月,平均(10.50±6.81)个月;临床表现为腹部肿物及血尿,病理组织形态特征为具有大核仁、丰富细胞质的横纹肌样细胞和经典嗜酸性细胞质的包涵体。免疫组织化学示肿瘤细胞质强表达波形蛋白,灶状表达细胞角蛋白、上皮膜抗原、分化簇99、分化簇34、肾母细胞瘤抑癌因子1、神经特异蛋白100、细胞周期素D1,不表达肿瘤抑制基因、结蛋白和肌浆蛋白。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 study the clinical and pathological features,treatment and prognosis of malignant rhabdoid tumor of kidney in children. Methods A retrospective analysis was conducted in the clinical data of 4 patients with malignant rhabdoid tumor of kidney from January 2006 to October 2019 in Kunming Children′s Hospital.Histological sections,light microscopy and immunohistochemical staining were performed. Results There were 2 males and 2 females in 4 cases of malignant rhabdoid tumor of kidney,they were 4-20 months with a average age of (10.50±6.81) months.The pathological features were rhabdoid cells with large nucleoli and abundant cytoplasm and inclusion bodies with classic eosinophilic cytoplasm.Immunohistochemistry showed that Vimentin was strongly expressed in the cytoplasm of tumor cells,and cytokeratin,epithelial membrane antigen,differentiation cluster 99,differentiation cluster 34,Wilms tumor suppressor 1,nerve specific protein 100,CyclinD1 were focal expressed,but tumor suppressor gene,Desmin and Myogenin were not expressed. Conclusion Malignant rhabdoid tumor of kidney in children is a highly aggressive malignant tumor.The tumor is easy to metastasize and relapse and has a poor prognosis.Radical nephrectomy plus chemotherapy is the main treatment.