Connecticut Children's Medical Center, Hartford, CT, USA.
Curr Urol Rep. 2013 Aug;14(4):350-8. doi: 10.1007/s11934-013-0330-0.
Wilms tumor represents the most common pediatric renal malignancy and the fourth most common childhood cancer overall. Overall survival from Wilms tumor has increased to over 90 % secondary to multidisciplinary therapy and multi-institutional cooperative group trials. Recent therapeutic focus has shifted to reduction in treatment morbidity and renal preservation while maintaining the high survival rates. Partial nephrectomy is an integral component of the multimodal treatment protocols for Wilms tumor patients with bilateral disease, solitary kidney, or predisposing syndromes. Recent consideration has been given to utilization of nephron sparing surgery (NSS) in carefully selected patients with nonsyndromic unilateral Wilms tumor. While long-term, prospective data in this subgroup of patients is not yet available, case series demonstrate comparable oncologic outcomes after partial versus radical nephrectomy. The relative rarity of Wilms tumor, especially those amenable to upfront partial nephrectomy, presents a challenge to conducting controlled trials.
威尔姆斯瘤是最常见的小儿肾恶性肿瘤，也是儿童期第四大常见的癌症。由于多学科治疗和多机构合作组试验，威尔姆斯瘤的总生存率已提高到 90%以上。最近的治疗重点已转移到减少治疗的发病率和保留肾脏，同时保持高生存率。对于双侧疾病、孤立肾或易患综合征的威尔姆斯瘤患者，部分肾切除术是多模式治疗方案的一个组成部分。最近，人们考虑在精心挑选的非综合征性单侧威尔姆斯瘤患者中使用保肾手术（NSS）。虽然这组患者的长期前瞻性数据尚不可用，但病例系列研究表明，部分肾切除术与根治性肾切除术的肿瘤学结果相当。威尔姆斯瘤相对罕见，尤其是那些适合 upfront 部分肾切除术的患者，这给进行对照试验带来了挑战。
