肾母细胞瘤合并肾母细胞瘤病6例临床分析  

Clinical analysis of six cases of nephroblastomatosis combined with Wilm＇s tumor

作　　者：宋宏程[1] 孙宁[1] 张潍平[1] 白继武[1] 田军[1] 谢向辉[1] 李明磊[1] 李宁[1] 黄澄如[1] 

机构地区：[1]首都医科大学附属北京儿童医院泌尿中心儿科学国家重点学科,100045

出　　处：《中华泌尿外科杂志》2011年第5期313-315,共3页Chinese Journal of Urology

基　　金：首都医学发展科研基金（2009-1045）

摘　　要：目的探讨肾母细胞瘤（wT）合并肾母细胞瘤病（Nbm）的诊治方法及预后。方法回顾分析2006年4月至2010年7月收治的6例wT合并Nbm患儿的临床资料。男4例，女2例；发病年龄5～14个月，平均9个月。wT位于左侧4例，右侧2例；Nbm位于WT同侧3例，对侧2例，双侧1例。wT均为单发，最大径分别为3、4、8、10、11、12cm，Nbm单发3例，多发3例。行瘤肾切除术2例，保留肾脏的肿瘤切除＋对侧肾上下极结节活检术1例，瘤肾切除十对侧保留肾脏的肿瘤切除术2例，保留肾脏的肿瘤及同侧结节切除术1例。术后以长春新碱、更生霉素或阿霉素化疗。结果1例术后化疗15个月，33个月后肿瘤复发，手术切除后5个月再次复发，继续化疗11个月后死于肝脏、肺部转移。4例术后化疗6个月，其中3例已停化疗分别9、12、21个月；1例拟化疗15个月，目前已化疗8个月。此5例目前未见肿瘤复发，仍在密切随访中。结论Nbm恶变率高，合并Nbm的wT复发率增高，化疗可降低Nbm恶变率，恶变或化疗无效的Nbm应行保留肾脏的肿瘤切除术。Objective To investigate the diagnosis, treatment and prognosis of nephroblastomatosis （Nbm） combined with Wilms, tumor （WT）. Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children＇s Hospital from 2006 to 2010 were reviewed retrospec tively. The patients＇ ages ranged from five to 14 months. Two of the patients were female and four were mate. The WTs were left-sided in four cases and right-sided in two cases. The Nbms were ipsi lateral with WT in three cases, contralateral in two cases and bilateral in one case. The Nbms were single in three cases and multiple in three cases. WTs were all single and the maximum diameter was 3, 4, 8, 10, 11, and 12 cm respectively. Two paitents underwent nephrectomy. Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases, Nephrectomy and contralaterai nephron sparing surgery was conducted in an additional two cases. Adjuvant chemotherapy included vincristine, actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy. Four patients underwent 6 months of chemotherapy, and it was 9, 12, and 21 months respectively after stop of chemo- therapy. Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT. Chemotherapy may reduce the rate of Nbm malignancy. If Nbm is malignant or chemotherapy is invalid, nephron sparing surgery is recommended.

关 键 词：肾母细胞瘤病 肾母细胞瘤 化疗 手术 

分 类 号：R737.11[医药卫生—肿瘤]