Objective: To further understand the clinical features, treatment efficacy and risk factors for poor prognosis in infantile-onset renal tumors. Methods: Clinical data of 45 cases of infantile-onset renal tumors from June 2011 to November 2019 in Peking University First Hospital, Beijing Children's Hospital, Beijing Tongren Hospital and Beijing Shijitan Hospital were analyzed retrospectively. The clinical features were summarized and the prognoses were evaluated. Multi-disciplinary diagnosis and treatment was used, including surgery, chemotherapy and radiotherapy. Kaplan-Meier analysis was used to calculate the overall survival rate and the event-free survival rate, while the chi-square test was used to analyze the risk factors for poor prognosis. Results: Among 45 patients, 24 were males and 21 females. The age of onset was 7 (ranged 3-11) months, and the length of tumor at initial diagnosis was 9.7 (ranged 4.9-25.0)cm. The International Society of Pediatric Oncology (SIOP) staging: 5 cases (11%) were in stage Ⅰ, 22 cases in stage Ⅱ (49%), 8 cases in stage Ⅲ (18%), 6 cases in stage Ⅳ (13%), and 4 cases in stage Ⅴ (9%). Risk groups included 5 cases (11%) in the low-risk group, 22 cases (49%) in the intermediate-risk group, and 18 cases (40%) in the high-risk group. Forty-four cases (98%) did not receive preoperative biopsy, 26 cases (58%) received preoperative chemotherapy, 39 cases (87%) received postoperative chemotherapy, and 2 cases (4%) received three-dimensional conformal radiotherapy. The 5-year overall survival rate was (83±7)%, and the 5-year event-free survival rate was (76±8)%. Hematuria as the first symptom (3/8 vs. 83% (30/36), χ²=7.005, P=0.024), tumor long diameter≤8 cm (5/11 vs. 85% (28/33), χ²=5.606, P=0.027) and high-risk pathological group (7/18 vs.100% (26/26), χ²=21.928, P<0.01) were risk factors for poor prognosis of children with renal tumors in this group. Conclusion: The prognosis of children with infantile-onset renal tumors is fairly well, nevertheless the prognosis is poor in patients with hematuria as the first symptom and in high-risk pathological group.
目的： 探讨婴儿期起病的肾脏肿瘤的临床特征、治疗效果和不良预后的高危因素。 方法： 回顾性分析2011年6月至2019年11月北京大学第一医院、北京儿童医院、北京同仁医院、北京世纪坛医院收治的婴儿期起病的45例肾脏肿瘤患儿的临床资料，总结临床特征并评估预后。治疗方法采用手术联合放化疗等多学科诊疗模式。预后分析采用Kaplan-Meier分析法计算总体生存率和无事件生存率，不良预后高危因素分析采用χ2检验。 结果： 45例患儿中男24例、女21例，起病年龄7（3~11）月龄，初诊时肿瘤长径9.7（4.9~25.0）cm。国际儿童肿瘤协会（SIOP）分期为Ⅰ期5例（11%），Ⅱ期22例（49%），Ⅲ期8例（18%），Ⅳ期6例（13%），Ⅴ期4例（9%）。危险度分组为低危组5例（11%），中危组22例（49%），高危组18例（40%）。44例（98%）未行术前穿刺活检，26例（58%）接受术前化疗，39例（87%）接受术后化疗，2例（4%）联合三维适型放疗。预计5年总体生存率（83±7）%，预计5年无事件生存率（76±8）%。以血尿为首发症状起病［3/8比83%（30/36），χ2=7.005，P=0.024］、肿瘤长径≤8 cm［5/11比 85%（28/33），χ2=5.606，P=0.027］以及病理类型划分为高危组［7/18比100%（26/26），χ2=21.928，P<0.01］是本组肾脏肿瘤患儿预后不良的高危因素。 结论： 婴儿期起病的肾脏肿瘤患儿预后良好，以血尿起病、高危组病理类型病例预后差。.