儿童肾母细胞瘤国际及国内诊治方案解读

Recent international and domestic advances in the diagnosis and treatment of Wilms tumor

肾母细胞瘤(Wilms tumor,WT)是儿童最常见的肾恶性肿瘤。COG(Children’s Oncology Group,儿童肿瘤协作组)和SIOP(International Society of Paediatric Oncology,国际儿童肿瘤学会)两大儿童肿瘤研究的国际协作组织分别形成了各自的诊疗方案:COG推荐手术优先,然后行化疗与放射治疗等辅助治疗;SIOP强调术前化疗,然后行手术与放射治疗。目前该两大协作组发布的肾母细胞瘤患者总体生存率均达到90%。中国抗癌协会小儿肿瘤专业委员会及中华医学会小儿外科学分会泌尿外科学组在COG和SIOP方案的基础上也先后发表了诊疗建议和共识。本文重点介绍COG方案和SIOP方案的异同,主要包括组织学分类、临床分期、危险度分组、治疗方案以及复发后治疗等方面;进而解读中国儿童肾母细胞瘤诊疗共识,分析中国的诊疗现状,供广大临床医师参考和借鉴。

Wilms tumor(WT)is the most common renal malignant tumor in children.This review was intended to present current standard processes of diagnosing and treating WT around the world.Two major international collaboration groups dedicated for the study of WT are Children's Oncology Group(COG,USA)and International Society of Paediatric Oncology(SIOP,Europe).COG has recommended primary surgery before any adjuvant treatment while SIOP trials tend to focus upon preoperative chemotherapy.Here the diagnostics,genomics and related syndromes of WT were summarized.Also histological classifications,risk stratifications,clinical stages and therapeutics of WT in COG/SIOP were analyzed.Current Chinese diagnosis and treatment consensus was discussed.Despite overall favorable survival outcomes of most WT children,further efforts should be made for minimizing late effects and formulating strategies for more accurate risk-stratified application of chemoradiotherapy.Surgeons must be more cognizant of the latest treatment protocols to achieve the most optimal outcomes for WT children.