Ko Edmund Y, Ritchey Michael L
Mayo Clinic College of Medicine, Phoenix, AZ, USA.
J Pediatr Urol. 2009 Feb;5(1):56-65. doi: 10.1016/j.jpurol.2008.08.007. Epub 2008 Oct 9.
Wilms' tumor is the most common renal tumor in children. Outcomes have improved dramatically over the past few decades, but important treatment questions remain. These include the role of molecular biologic markers in stratifying patients for therapy or targeting tumors for treatment. We present a summary of these advances and outline the current treatment of Wilm's tumor.
The medical literature and results of all cooperative group studies reporting treatment of children with Wilms' tumor were reviewed.
Overall survival exceeds 90% for most patients with nephroblastoma. However, outcomes for patients with rhabdoid tumors and diffuse anaplasia remain poor. The role of renal sparing surgery in patients with bilateral tumors is clear, but for children with unilateral tumors it continues to be defined.
Current protocols conducted by pediatric oncology groups are beginning to incorporate biologic features to stratify patients for therapy. Treatment strategies continue to focus on limiting late effects of treatment while maintaining an excellent survival. New therapies are needed to treat the high-risk patients who continue to have high relapse and mortality rates.
肾母细胞瘤是儿童最常见的肾脏肿瘤。在过去几十年中，其治疗结果有了显著改善，但仍存在一些重要的治疗问题。这些问题包括分子生物学标志物在对患者进行治疗分层或针对肿瘤进行靶向治疗中的作用。我们总结了这些进展，并概述了目前肾母细胞瘤的治疗方法。
回顾了所有协作组研究中关于儿童肾母细胞瘤治疗的医学文献及结果。
大多数肾母细胞瘤患者的总生存率超过90%。然而，横纹肌肉瘤和弥漫性间变患者的治疗结果仍然较差。保留肾手术在双侧肿瘤患者中的作用是明确的，但对于单侧肿瘤患儿，其作用仍有待确定。
儿科肿瘤学组实施的当前方案开始纳入生物学特征以对患者进行治疗分层。治疗策略继续侧重于在保持高生存率的同时限制治疗的晚期效应。需要新的疗法来治疗那些复发率和死亡率仍然较高的高危患者。
