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Table 1. Syndromes and Conditions Associated With Wilms Tumor

Syndrome/Condition  Gene Overgrowth Phenotype  Nonovergrowth Phenotype 
WAGR = Wilms tumor, aniridia, genitourinary anomaly, and mental retardation.
9q22.3 microdeletion syndrome9q22.3X
Beckwith-Wiedemann syndromeWT2 X
Isolated hemihypertrophyX
Perlman syndromeDIS3L2 X
Simpson-Golabi-Behemel syndromeGPC3 X
Sotos syndromeNSD1 X
Bloom syndromeBLM X
Denys-Drash syndromeWT1 X
Familial Wilms tumorFWT1 X
Frasier syndromeWT1 X
Genitourinary anomaliesWT1 X
Fanconi anemia with biallelic mutations in BRCA2 (FANCD1) or PALB2 (FANCN)BRCA2 X
Li-Fraumeni syndromeTP53 X
Sporadic aniridiaWT1 X
Trisomy 18X
WAGR syndromeWT1 X