【摘要】 背景与目的肾脏恶性肿瘤是儿童常见的并可治愈的恶性疾病,本文总结了在上海儿童医学中心血液/肿瘤科治疗的肾脏恶性肿瘤患者的临床特点和治疗情况,评估“WT-99”方案的耐受性和疗效。方法自1998年10月至2005年7月,我院血液/肿瘤科收治的初治的肾脏恶性肿瘤患儿42例,按病理类型分成FH组、UFH组和肾肉瘤组,采用“WT-99”方案、采取联合治疗的模式、并根据病理类型进行分期分治。结果至2005年11月1日,35/42例存活(83.3%)并在随访中,3例患儿在缓解期失访,中位随访时间31个月(范围4~85个月)。FH组和肾肉瘤组的5年EFS分别为(87.10±7.24)%(n=32)和(83.33±15.21)%(n=7);UFH组4年EFS为(66.67±27.22)%(n=3)。共5例复发,1例再治后获第二次缓解,2例复发后未治失访,2例死于疾病进展。本组病例无治疗相关性死亡,亦无严重的治疗相关并发症发生。结论“WT-99”方案可有效治疗儿童肾脏恶性肿瘤,并且耐受性良好,值得推广。更多还原

【Abstract】 Background and purpose:Renal malignancies are one of the most common tumor in childhood and are curable. In this article we analyse the clinical features of childhood renal malignancies and evaluate the effect of ’WT-99’ Regimen in terms of tumor response and toxicities. Methods:From Oct 1st 1998 to July 31st 2005, 42 eligible patients with newly diagnosed renal malignancies were admitted. 35/42 pts were Wilm’s tumor, 32/35 had FH histology (FH group), 3/35 had UFH histology (UFH group). 7/42 had renal sarcomas (sarcoma group). The overall male-to-female ratio was 1.6:1. The median age was 2.42 years old. All of the patients were treated with WT-99 Regimen based on the National Wilm’s Tumor Study Group’s Regimen.Results:Till Nov. 1st, 2005, 35/42 (83.3%) patients survived, The median follow-up time of 42 patients was 31 months (range: 4-85 moths). 7/42 patients were lost during follow up. The 5 year EFS in FH group, UFH group(4 year EFS), and sarcoma group were 87.10±7.24%(n=32), 66.67±27.22%(n=3)and 83.33±15.21%(n=7), respectively(χ 2 =1.44,P=0.49)). 5 patients relapsed, the median relapse time from diagnosis was 9.6 months (range: 2~24 months). 1/5 got CR2 after salvage therapy. There were no therapy related death and serious side effects in terms of secondary malignancies or cardiac dysfunctions.Conclusions:Renal malignancy is one of the most common malignant tumors in childhood. It can be cured after the treatment according to different stages and histology by multi-disciplinary therapy. Our experience showed that ’WT-99’ Regimen is effective and well tolered.更多还原