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目的回顾性分析预后良好型Ⅲ~Ⅳ期肾母细胞瘤的临床特点、治疗效果和预后因素,为进一步优化治疗方案提供临床依据。方法收集和分析收治的年龄≤15岁的初治预后良好型Ⅲ~Ⅳ期肾母细胞瘤患者的临床资料,采用SPSS 23.0统计软件对生存资料进行分析。用Kaplan-Meier法计算生存率。结果共入组肿瘤分期为Ⅲ~Ⅳ期的病例87例。男∶女为41∶46;中位年龄2岁。左肾48例,右肾39例;Ⅲ期68例,Ⅳ期19例。病理组织类型:上皮型10例、间质型9例、胚基型15例、混合型42例、预后良好型未分亚型11例。44例患者一开始接受手术切除;43例患者先接受新辅助化疗后再手术。术后58例患者接受了放疗(67.0%)。手术前后进行共24周化疗。中位随访时间33个月,进展4例,复发7例,第二肿瘤2例,共9例死亡。全组5年总生存率为87.1%,其中Ⅲ期为91.9%,Ⅳ期为71.1%;全组5年无事件生存率为83.5%,其中Ⅲ期为89.4%,Ⅳ期为62.2%。临床分期是总生存(P=0.006)和无事件生存(P=0.005)的独立预后因素。结论本研究Ⅲ~Ⅳ期儿童青少年预后良好型肾母细胞瘤总生存与国外发达国家相当,Ⅳ期无事件生存率低于国外,应完善危险度分层,优化治疗方案,提高生存率,降低治疗毒性不良反应。
Objective To retrospectively analyze the clinical features, treatment effects and prognostic factors of stage Ⅲ ~ Ⅳ advanced nephroblastoma, so as to provide a clinical basis for further optimization of treatment options. Methods The clinical data of patients with newly diagnosed stage III-IV nephroblastoma who underwent treatment were collected and analyzed. Survival data were analyzed using SPSS 23.0 statistical software. Survival was calculated using Kaplan-Meier method. Results A total of 87 cases with stage Ⅲ ~ Ⅳ tumors were included in the study. Male: Female 41:46; median age 2 years old. 48 cases of left kidney, 39 cases of right kidney; 68 cases of stage Ⅲ, 19 cases of stage Ⅳ. Pathological types: epithelial type in 10 cases, interstitial in 9 cases, embryo in 15 cases, mixed type in 42 cases, with good prognosis in 11 cases. Forty-four patients were initially surgically removed; 43 received neoadjuvant chemotherapy prior to surgery. Postoperative 58 patients received radiotherapy (67.0%). A total of 24 weeks of chemotherapy before and after surgery. The median follow-up time was 33 months, with 4 cases of progression, 7 cases of recurrence, 2 cases of the second tumor and 9 cases of death. The overall 5-year overall survival rate was 87.1%, of which 91.9% in stage III and 71.1% in stage IV. The 5-year event-free survival rate was 83.5% in all patients, with stage III of 89.4% and stage IV of 62.2%. Clinical stage was an independent prognostic factor for overall survival (P = 0.006) and event-free survival (P = 0.005). Conclusion The prognosis of stage Ⅲ ~ Ⅳ children and adolescents with good prognosis of nephroblastoma is comparable to that of developed countries. The survival rate of stage Ⅳ is lower than that of other countries. The risk stratification should be improved to optimize the treatment plan to improve the survival rate and reduce Treat toxic side effects.