Sawicz-Birkowska Krystyna, Czernik Jerzy, Bagłaj Maciej, Czauderna Piotr, Kantorowicz-Szymik Sabina, Poznański Wojciech A, Mańkowski Przemysław, Madziara Wojciech, Prokurat Andrzej, Osemlak Jerzy
Klinika Chirurgii i Urologii Dzieciecej Akademii Medycznej we Wrocławiu.
Przegl Lek. 2004;61 Suppl 2:20-3.
Nephroblastoma is the most common kidney tumor in Polish children.
to present clinical material and outcome of 533 children with renal tumors.
500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002. Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts. All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months. Pre-operative chemotherapy was done to 93.8% pts.
Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma. Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%). In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed. Surgical complications were mild and occurred only in 8.9% pts.
5-years overall survival of CS I pts (favorable and standard histology) is 93.48%, CS II--96.8%, CS III--84.4%, CS IV--67%, CS V--58%. The results of treatment of 33 pts with non-Wilms renal tumors have improved lately. 78.7% of our pts achieved 5-years overall survival.
The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children. The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.
肾母细胞瘤是波兰儿童中最常见的肾脏肿瘤。
介绍533例肾肿瘤患儿的临床资料及治疗结果。
登记了500例肾母细胞瘤患儿和33例非威尔姆斯肿瘤患儿:包括先天性中胚层肾瘤、肾细胞癌、先天性肾囊肿、肾胚胎瘤及其他肿瘤,1993年至2002年间患儿平均年龄4.5岁。分期:I期——148例,II期——191例,III期——114例,IV期——51例,V期——29例。所有肾母细胞瘤患儿均按照首个国家PPGGL 01 - 92方案进行治疗,6个月以上I - III期患儿术前化疗采用放线菌素D(ACT)、长春新碱(VCR),IV期患儿采用ACT、VCR、阿霉素(DOX)。93.8%的患儿进行了术前化疗。
6个月以上的451例(98%)肾母细胞瘤患儿及44例(8.2%)6个月以下婴儿在术前化疗后接受了根治性肾切除术。6例(1.2%)单侧肿瘤患儿在术前化疗后接受了部分肾切除术。29例V期肾母细胞瘤中有26例(89.65%)可行保肾手术,12例进行了单侧肾切除术。手术并发症轻微,仅8.9%的患儿出现。
I期(组织学类型良好和标准)患儿的5年总生存率为93.48%,II期——96.8%,III期——84.4%,IV期——67%,V期——58%。33例非威尔姆斯肾肿瘤患儿的治疗结果近来有所改善。我们的患儿中有78.7%实现了5年总生存。
根据国际小儿肿瘤学会(SIOP)肾母细胞瘤方案(01 - 92)的建议,对所有6个月以上患儿使用全身新辅助化疗可使肿瘤缩小,便于完整的手术切除肾脏,对儿童肾肿瘤治疗非常有利。由于引入了新的、更积极的化疗方案,非威尔姆斯肿瘤的治疗结果也有所改善。