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

Incidence

The incidence of Wilms tumor is 7.1 cases per 1 million children younger than 15 years. Approximately 500 cases of Wilms tumor are diagnosed in the United States each year. The incidence is substantially lower in Asians. The male to female ratio in unilateral cases of Wilms tumor is 0.92:1.00, but in bilateral cases it is 0.60:1.00. The mean age at diagnosis is 44 months in unilateral cases of Wilms tumor and 31 months in bilateral cases.[1,2] About 10% of children with Wilms tumor have an associated congenital malformation syndrome.[3]

Syndromes and Other Conditions Associated With Wilms Tumor

Wilms tumor typically develops in otherwise healthy children; however, approximately 10% of children with Wilms tumor have been reported to have a congenital anomaly.[3,4] Of 295 consecutive patients with Wilms tumors seen at the Institute Curie in Paris, 52 (17.6%) had anomalies or syndromes, 43 of which were considered major, and 14 of which were genetically proven tumor predisposition syndromes.[5] Children with Wilms tumors may have associated hemihypertrophy and urinary tract anomalies, including cryptorchidism and hypospadias. Children may have a recognizable phenotypic syndrome (including overgrowth, aniridia, genetic malformations, and others). These syndromes have provided clues to the genetic basis of the disease. The phenotypic syndromes and other conditions have been grouped into overgrowth and nonovergrowth categories. Overgrowth syndromes and conditions are the result of excessive prenatal and postnatal somatic growth.[6,7]

Table 1. Syndromes and Conditions Associated With Wilms Tumor

Syndrome/ConditionGeneOvergrowth PhenotypeNonovergrowth Phenotype
WAGR = Wilms tumor, aniridia, genitourinary anomaly, and mental retardation.
9q22.3 microdeletion syndrome9q22.3X 
Beckwith-Wiedemann syndromeWT2X 
Isolated hemihypertrophy X 
Perlman syndromeDIS3L2X 
Simpson-Golabi-Behemel syndromeGPC3X 
Sotos syndromeNSD1X 
Bloom syndromeBLM X
Denys-Drash syndromeWT1 X
Familial Wilms tumorFWT1 X
FWT2
Frasier syndromeWT1 X
Genitourinary anomaliesWT1 X
Fanconi anemia with biallelic mutations inBRCA2(FANCD1) orPALB2(FANCN)BRCA2 X
PALB2
Li-Fraumeni syndromeTP53 X
CHEK2
Sporadic aniridiaWT1 X
Trisomy 18  X
WAGR syndromeWT1 X

For information about the genes associated with Wilms tumor, including Wilms tumor 1 (WT1) and Wilms tumor 2 (WT2), refer to the Genes Associated With Wilms Tumor section of this summary.

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