Establishment of new therapeutic protocol for pediatric renal tumor according to the new risk classification

我们分析了84例肾母细胞瘤中抑癌基因RASSF 1A的甲基化状态，发现它与不良预后显著相关。此外，我们分析了WTX基因的突变，发现这也与不良结局显著相关。因此，这些生物标志物被认为是风险分类的新生物标志物的候选者，以创建新的治疗方案。双侧肾母细胞瘤（BWT）的生存率已得到提高，但在肾脏保护方面的结果并不令人满意。我们回顾了在JWiTS中注册的BWT的结果。仅10/28例（36%）患者行双侧肾单位保留手术（NSS），10例（40%）患儿发生肾功能损害。应改变方案以提高肾实质保留率。术前应进行化疗以缩小肿瘤，随后应在中心影像学评估后进行NSS。

We analyzed methylation status of tumor suppressor gene RASSF1A in 84 Wilms tumors and found out that it was significantly associated with a poor outcome. Moreover, we analyzed the mutation of WTX gene and revealed that this also was significantly associated with a poor outcome. Therefore these biomarkers were considered be candidates of the new biomarkers for risk classification to create the new therapeutic protocols.The survival rate of bilateral Wilms tumors (BWT) has been improved; however, the results are not satisfactory in terms of the renal preservation. We reviewed the outcome of BWT registered in JWiTS. Bilateral nephron-sparing surgery (NSS) was achieved only in 10 of 28 cases (36%) and 10 children (40%) developed impaired renal function. The protocol should be changed to improve the rate of preservation of the renal parenchyma. Preoperative chemotherapy should be performed to shrink the tumors, and subsequent NSS should be carried out after a central imaging evaluation.