Wilms tumor

Wilms tumor is the most common renal malignancy in children.

Usually presents as a unilateral, painless, abdominal/flank mass; less commonly presents bilaterally.

Metastatic disease occurs in <10% of patients and needs to be carefully excluded using ultrasound and computed tomography/magnetic resonance imaging.

Long-term survival approaches 90% in localized disease.

Treatment is with nephrectomy, chemotherapy, and radiation therapy.

Wilms tumor, or nephroblastoma, is the most common form of renal malignancy in childhood.[1]Nakata K, Colombet M, Stiller CA, et al. Incidence of childhood renal tumours: an international population-based study. Int J Cancer. 2020 Dec 15;147(12):3313-27. https://onlinelibrary.wiley.com/doi/10.1002/ijc.33147 http://www.ncbi.nlm.nih.gov/pubmed/32902866?tool=bestpractice.com It usually occurs in the first 5 years of life.[2]Szychot E, Apps J, Pritchard-Jones K. Wilms' tumor: biology, diagnosis and treatment. Transl Pediatr. 2014 Jan;3(1):12-24. https://tp.amegroups.com/article/view/3228/html http://www.ncbi.nlm.nih.gov/pubmed/26835318?tool=bestpractice.com The risk for developing Wilms tumor is increased in certain congenital overgrowth syndromes, congenital nonovergrowth syndromes, and children with congenital urogenital anomalies.[3]Spreafico F, Fernandez CV, Brok J, et al. Wilms tumour. Nat Rev Dis Primers. 2021 Oct 14;7(1):75. https://www.nature.com/articles/s41572-021-00308-8 http://www.ncbi.nlm.nih.gov/pubmed/34650095?tool=bestpractice.com Long-term survival approaches 90% in localized disease.[2]Szychot E, Apps J, Pritchard-Jones K. Wilms' tumor: biology, diagnosis and treatment. Transl Pediatr. 2014 Jan;3(1):12-24. https://tp.amegroups.com/article/view/3228/html http://www.ncbi.nlm.nih.gov/pubmed/26835318?tool=bestpractice.com