Screening for susceptibility syndromes associated with Wilms tumor in children and recent thera-peutic advances
Wilms tumor(WT)is one of the most common malignancies in children.It accounts for around 7%of pediatric tumors.And 10%-17%of WTs are associated with cancer predisposition syndrome(CPS)with an earlier onset,a greater malignancy and a higher incidence.Currently survival rate of WT in non-susceptible syndrome is over 90%.Treatment options include surgery plus chemoradiotherapy.Success rate of WT in susceptible syndrome is low and treatments and outcomes vary.The associated susceptibility syndromes include WT-Aniridia-Genitourinary Anomalies-Mental Retardation(WAGR),Denys-Drash and Beckwith-Wiedemann.Currently domestic physicians lack a basic understanding of treating and preventing WT-related syndromes.This review focused upon screening for susceptibility syndromes associated with WT and its proper managements in children.