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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information


In an analysis of Wilms tumor patients in the Surveillance, Epidemiology and End Results database, adults (n = 152) had a statistically worse overall survival (OS) (69% vs. 88%, P < .001) than pediatric patients (n = 2,190), despite previous studies showing comparable outcome when treated on protocol.[9,10] Adults with Wilms tumor were more likely than pediatric patients to be staged as having localized disease, to not receive any lymph node sampling, and to not receive any radiation treatment. The investigators recommended that all adult patients diagnosed with Wilms tumor should undergo lymph node sampling and that there should be close collaboration with pediatric surgeons and oncologists in treatment planning. The Children's Oncology Group has increased the enrollment age for their Wilms tumor trials to include patients up to age 30 years.[11]

Congenital Anomalies and Syndromes Predisposing to Wilms Tumor

Wilms tumor typically develops in otherwise healthy children; however, approximately 10% of children with Wilms tumor have a congenital anomaly.[12] Children with Wilms tumor may have associated urinary tract anomalies, including hemihypertrophy, cryptorchidism, and hypospadias. Children may have a recognizable phenotypic syndrome (including overgrowth disease, aniridia, genetic malformations, and others). These syndromes have provided clues to the genetic basis of the disease. The phenotypic syndromes have been divided into overgrowth and nonovergrowth categories.

  • Overgrowth syndromes. Overgrowth syndromes are the result of excessive prenatal and postnatal somatic growth.[13,14] Examples of overgrowth syndromes include the following:
    • Beckwith-Wiedemann syndrome (prevalence is about 1% of children with Wilms tumor).[15,16,17,18]
    • Isolated hemihypertrophy (prevalence is about 2.5% of children with Wilms tumor).[15,19]
    • Perlman syndrome (characterized by fetal gigantism, renal dysplasia, Wilms tumor, islet cell hypertrophy, multiple congenital anomalies, and mental retardation).[20] Germline inactivating mutations in DIS3L2 on chromosome 2q37 are associated with Perlman syndrome.[21]
    • Sotos syndrome (characterized by cerebral gigantism).
    • Simpson-Golabi-Behemel syndrome (characterized by macroglossia, macrosomia, renal and skeletal abnormalities, and increased risk of embryonal cancers).
  • Nonovergrowth syndromes. Examples of nonovergrowth syndromes associated with Wilms tumor include the following:
    • WAGR syndrome (aniridia, genitourinary anomaly, and mental retardation). The constellation of WAGR syndrome occurs in association with an interstitial deletion on chromosome 11 (del[11p13]).[22,23] (prevalence is about 0.4% of children with Wilms tumor). The incidence of bilateral Wilms tumor in children with WAGR syndrome is about 15%.[24]
    • Isolated aniridia.
    • Genitourinary anomalies including hypospadias, undescended testis, and others are associated with Wilms tumor 1 (WT1) mutations (prevalence is over 6% of children with Wilms tumor). Children with pseudo-hermaphroditism and/or renal disease (glomerulonephritis or nephrotic syndrome) who develop Wilms tumor may have the Denys-Drash or Frasier syndrome (characterized by male hermaphroditism, primary amenorrhea, chronic renal failure, and other abnormalities),[25] both of which are associated with mutations in the WT1 gene.[26] Specifically, germline missense mutations in the WT1 gene are responsible for most Wilms tumors that occur as part of the Denys-Drash syndrome.[27,28]
    • Bloom syndrome.
    • Alagille syndrome.[29]
    • Trisomy 18.
    • Li-Fraumeni syndrome (familial cancer syndrome).
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