Wilms' tumour

Most common renal malignancy in children.

Presents as a unilateral, painless, abdominal/flank mass; rarely presents bilaterally.

Metastatic disease occurs in <10% of patients and needs to be carefully excluded using ultrasound and CT/MRI.

Long-term survival approaches 90% in localised disease.

Treatment is with nephrectomy, chemotherapy, and radiotherapy.

Wilms' tumour, or nephroblastoma, is the most common form of renal malignancy in childhood. It usually occurs in the first 2 to 5 years of life.[1]D'Angio GJ. The National Wilms Tumor Study: a 40 year perspective. Lifetime Data Anal. 2007 Dec;13(4):463-70. http://www.ncbi.nlm.nih.gov/pubmed/18027087?tool=bestpractice.com [2]Kaste SC, Dome JS, Babyn PS, et al. Wilms tumour: prognostic factors, staging, therapy and late effects. Pediatr Radiol. 2008 Jan;38(1):2-17. http://www.ncbi.nlm.nih.gov/pubmed/18026723?tool=bestpractice.com Approximately 500 children and adolescents <20 years of age are diagnosed each year in the US.[3]Breslow N, Olshan A, Beckwith JB, et al. Epidemiology of Wilms tumor. Med Pediatr Oncol. 1993;21(3):172-81. http://www.ncbi.nlm.nih.gov/pubmed/7680412?tool=bestpractice.com [4]Bunin GR, Meadows AT. Epidemiology and Wilms tumor: approaches and methods. Med Pediatr Oncol. 1993;21(3):169-71. http://www.ncbi.nlm.nih.gov/pubmed/8383277?tool=bestpractice.com The risk for developing Wilms' tumour is increased in certain congenital overgrowth syndromes, congenital non-overgrowth syndromes, and children with congenital urogenital anomalies.[5]Green DM, Breslow NE, Beckwith JB, et al. Screening of children with hemihypertrophy, aniridia, and Beckwith-Wiedemann syndrome in patients with Wilms tumor: a report from the National Wilms Tumor Study. Med Pediatr Oncol. 1993;21(3):188-92. http://www.ncbi.nlm.nih.gov/pubmed/8095320?tool=bestpractice.com [6]Heppe RK, Koyle MA, Beckwith JB. Nephrogenic rests in Wilms tumor patients with the Drash syndrome. J Urol. 1991 Jun;145(6):1225-8. http://www.ncbi.nlm.nih.gov/pubmed/1851891?tool=bestpractice.com [7]Breslow NE, Norris R, Norkool PA, et al. Characteristics and outcomes of children with the Wilms tumor-Aniridia syndrome: a report from the National Wilms Tumor Study Group. J Clin Oncol. 2003 Dec 15;21(24):4579-85. http://www.ncbi.nlm.nih.gov/pubmed/14673045?tool=bestpractice.com [8]Mahmood A, Ghafoor T, Badsha S. Wilms' tumour: presentation and treatment. J Coll Physicians Surg Pak. 2004 Mar;14(3):142-5. http://www.ncbi.nlm.nih.gov/pubmed/15228845?tool=bestpractice.com [9]Breslow NE, Olson J, Moksness J, et al. Familial Wilms' tumor: a descriptive study. Med Pediatr Oncol. 1996 Nov;27(5):398-403. http://www.ncbi.nlm.nih.gov/pubmed/8827065?tool=bestpractice.com [10]van den Heuvel-Eibrink MM, Grundy P, Graf N, et al. Characteristics and survival of 750 children diagnosed with a renal tumor in the first seven months of life: a collaborative study by the SIOP/GPOH/SFOP, NWTSG, and UKCCSG Wilms tumor study groups. Pediatr Blood Cancer. 2008 June;50(6):1130-4. http://www.ncbi.nlm.nih.gov/pubmed/18095319?tool=bestpractice.com Long-term survival approaches 90% in localised disease.[Figure caption and citation for the preceding image starts]: Resected Wilms' tumourUniversity of Utah Health Sciences Center, Salt Lake City, Utah; used with permission [Citation ends].