Wilms' tumor: biology, diagnosis and treatment
Wilms' tumor: biology, diagnosis and treatment
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DOI：
10.3978/j.issn.2224-4336.2014.01.09
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发表时间：
2014-01-01
影响因子：
2
通讯作者：
Pritchard-Jones, Kathy
中科院分区：
文献类型：
--
作者：
Szychot, Elwira;Apps, John;Pritchard-Jones, Kathy
肾母细胞瘤是儿童期最常见的肾脏肿瘤，每10，000名儿童中就有一名患有肾母细胞瘤。它也是儿科肿瘤学的成功之一，局部疾病的长期生存率超过90%，转移性疾病的长期生存率超过75%。肾母细胞瘤的成功治疗需要仔细注意肿瘤的正确分期，以及儿科肿瘤学家，专科外科医生，放射科医生，病理学家和放射肿瘤学家之间的合作。虽然目前的治疗方案是基于风险分配，以最大限度地减少低风险患者的毒性和改善高风险疾病的结局，但在识别新的分子，组织学和临床风险因素用于治疗强度分层方面仍然存在挑战。关于Wilms肿瘤生物学和治疗的知识正在迅速发展，并且仍然是多模式恶性肿瘤治疗的范例。未来的努力将集中在使用生物标志物来改善风险分层和引入新的分子靶向治疗，这将最大限度地减少毒性并改善组织学不良和复发性疾病患者的结局。本文的目的是总结我们对肾母细胞瘤生物学的认识，并描述目前的临床治疗方法。
Wilms' tumor is the commonest renal tumor of childhood affecting one in 10,000 children. It is also one of the successes of paediatric oncology with long term survival above 90% for localised disease and 75% for metastatic disease. Successful management of Wilms' tumor necessitates meticulous attention to correct staging of the tumor and a collaborative effort between paediatric oncologists, specialist surgeons, radiologists, pathologists, and radiation oncologists. Although current treatment protocols are based on risk assignment to minimise toxicity for low risk patients and improve outcomes for those with high risk disease, challenges remain in identifying novel molecular, histological and clinical risk factors for stratification of treatment intensity. Knowledge about Wilms' tumor biology and treatment is evolving rapidly and remains a paradigm for multimodal malignancy treatment. Future efforts will focus on the use of biomarkers to improve risk stratification and the introduction of newer molecularly targeted therapies that will minimise toxicity and improve the outcomes for patients with unfavourable histology and recurrent disease. The aim of this article is to summarise advances in our understanding of the biology of Wilms' tumor and to describe the current approaches to clinical management of patients.