Summary
Nephroblastoma or Wilms tumor is the most frequent renal tumor in children, accounting for 6 to 8% of pediatric cancers. Over 80% occur before the age of 5 years. Nephroblastomas may be associated with a variety of congenital malformations (aniridia, hemihypertrophy, genitourinary defects) or be part of specific syndromes (Beckweth-Widemann syndrome, Denys-Drash syndrome, WAGR or Perlman's syndromes). Loss of heterozygosity of chromosome 11 has led to the identification of several genes involved in the genesis of these tumors. Nephroblastoma has a strong tendency to metastasize in lungs, liver, retroperitoneal space and peritoneal cavity in case of tumor effraction, and in lymph nodes and bones. This is a very chemosensitive tumor. Patients are first treated with anticancer drugs to eradicate disseminated disease and reduce the size of the primary tumor, making surgery easier. Then complete surgery is performed without tumor effraction. Chemotherapy varies in type and in duration according to histopathology and staging. Radiation therapy only applies to forms with poor prognosis. Survival is over 90%. In some cases recurrences can be cured with specific treatments. Patient management requires a pluridisciplinary team with expertise in the field of pediatric cancer. * Author: Pr. G. Vassal (January 2002) *
