Wilms tumor, or nephroblastoma, represents about 90% of all pediatric renal tumors and about 7% of all pediatric malignancies. Most Wilms tumors are unilateral, although in 5-10 % of the patients both kidneys are infected. Wilms tumor typically occurs between the age of 2 and 4 years, and 90% of the patients are diagnosed before the age of 7 years. Above the age of 18 years, Wilms tumor is rare, representing less than 1% of all adult renal tumors. Most pediatric Wilms tumor patients present with an asymptomatic abdominal mass, although abdominal pain, anorexia, vomiting, hematuria, fever and hypertension have frequently been described. Unusual presentations of Wils tumor in children are acquired von Willebrand disease, sudden death due to pulmanory embolism, and Cushing syndrome.

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Erasmus University Rotterdam, MRC Holland, Novartis Oncology, Pediatric Oncology Foundation Rotterdam (KOCR), Stichting Juul, Takeda Nederland BV, The Sophia Foundation for Medical Research (SSWO)
R. Pieters (Rob)
Erasmus University Rotterdam
Erasmus MC: University Medical Center Rotterdam

Segers, H. (2013, September 12). Wilms tumors: genotypes and phenotypes. Retrieved from http://hdl.handle.net/1765/41245