儿童肾脏原发非Wilms瘤恶性肿瘤的临床特点  

Clinical features of children with primary malignant non-Wilms renal tumors

作　　者：焦瑒瑒 付盼 杨静薇 蒋莎义 廖雪莲 邵静波[1] JIAO Yangyang;FU Pan;YANG Jingwei;JIANG Shayi;LIAO Xuelian;SHAO Jingbo(Department of Hematology and Oncology,Shanghai Children's Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai 200062,China)

机构地区：[1]上海市儿童医院,上海交通大学医学院附属儿童医院血液肿瘤科,上海200062

出　　处：《现代肿瘤医学》2023年第4期702-706,共5页Journal of Modern Oncology

基　　金：上海市科委自然科学基金项目(编号:19ZR1442400)。

摘　　要：目的:探讨儿童肾脏原发非Wilms瘤恶性肿瘤的临床特点及预后。方法:回顾性分析我院血液肿瘤科2015年1月至2020年12月收治的22例儿童肾脏原发非Wilms瘤恶性肿瘤的临床资料。结果:本组共22例儿童肾脏原发非Wilms瘤恶性肿瘤,男性12例,女性10例,中位发病年龄42.5月(4月~173月),肾透明细胞肉瘤10例,肾恶性横纹肌样瘤5例,肾细胞癌4例,神经母细胞瘤2例,尤文肉瘤1例,临床表现以肉眼血尿和体格检查发现腹部包块最常见,临床分期:Ⅰ期2例,Ⅱ期3例,Ⅲ期11例,Ⅳ期6例,21例患者行根治性肿瘤切除术,随访时间23月(4~80月),2年和5年OS分别为81.3%和69.7%,临床分期Ⅳ期OS和EFS较临床分期Ⅰ+Ⅱ+Ⅲ期患者低,差异有统计学意义(P<0.05)。结论:儿童肾脏原发非Wilms瘤恶性肿瘤临床罕见,不同类型肿瘤的诊断主要依据免疫组化及分子诊断,临床分期Ⅳ期与预后不良相关,早期发现、早期多学科联合诊治有助于改善预后。Objective:To investigate the clinical features and prognosis of primary non-Wilms renal tumors in children.Methods:A retrospective analysis was performed on 22 cases with primary non-Wilms renal tumors in department of hematology and oncology of Shanghai Children’s Hospital from January 2015 to December 2020.Results:Totally 22 patients were included in the study, including 12 males and 10 females, with a median age of 42.5 months(4~173 months).10 cases were clear cell sarcoma.5 cases were malignant rhabdoid tumor.4 cases were renal cell carcinoma.2 cases were neuroblastoma and 1 case was ewings sarcoma.The common clinical manifestations were hematuresis and mass found in physical examination.2 cases were in stage Ⅰ,3 cases in stage Ⅱ,11 cases in stage Ⅲ,and 6 cases in stage Ⅳ.Radical tumor resection was performed on 21 patients.The median follow-up time was 23 months(4~80 months).The 2-year and 5-year OS rate were 81.3% and 69.7% respectively.The OS rate of stage Ⅳ was lower than stage Ⅰ+Ⅱ+Ⅲ and the difference was statistically significant.Conclusion:The primary non-Wilms renal tumors are rare.Immunohistochemistry and molecular diagnostic approaches are of considerable importance to establish a precise diagnosis.Stage Ⅳ is associated with poor prognosis.Early detection, diagnosis and treatment of early multidisciplinary can help improve prognosis.

关 键 词：儿童 肾脏恶性肿瘤 非Wilms瘤 预后 

分 类 号：R737.11[医药卫生—肿瘤]