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

    Table 1. Syndromes and Conditions Associated With Wilms Tumor continued...

    Nonsyndromic causes of Wilms tumor

    Nonsyndromic causes of Wilms tumor include the following:

    • Familial Wilms tumor. Despite the number of genes that appear to be involved in the development of Wilms tumor, familial Wilms tumor is uncommon, with approximately 2% of patients having a positive family history for Wilms tumor. Siblings of children with Wilms tumor have a less than 1% chance of developing Wilms tumor.[28,29,30] The risk of Wilms tumor among offspring of persons who have had unilateral (sporadic) tumors is less than 2%.[31]

      Two familial Wilms tumor genes have been localized to FWT1 (17q12-q21) and FWT2 (19q13.4).[32,33,34] There are occasional Wilms tumor families with a germline mutation in WT1. In these families, most, but not all, family members have genitourinary tract malformations.[35,36]

    • WT1-related.
      • Sporadic aniridia. Sporadic aniridia may result from small germline deletions of one copy of the PAX6 gene that includes part or all of the adjacent WT1 gene but does not result in genitourinary abnormalities or retardation (i.e., not obviously WAGR syndrome). Therefore, many patients with sporadic aniridia develop Wilms tumors and are candidates for screening. The relative risk of Wilms tumor in sporadic aniridia is 67-fold.[37] About half of individuals with sporadic aniridia and PAX6 and WT1 deletions develop Wilms tumor.[38]
    • Isolated hemihypertrophy. Hemihypertrophy is an asymmetric overgrowth of one or more body parts and is associated with Wilms tumor. It can also be associated with other predisposition syndromes such as Beckwith-Wiedemann syndrome. Clinical signs may not be very evident, and hemihypertrophy may be noted after tumor diagnosis. The overall Wilms tumor incidence was 5.9% in a study of 168 patients with isolated hemihypertrophy.[39] The prevalence is about 2.5% of children with Wilms tumor.[15,39]
    • Trisomy 18.[40]
    • Fanconi anemia with biallelic mutations in BRCA2 (FANCD1) or PALB2 (FANCN). BRCA2 and PALB2 play central roles in homologous recombination DNA repair. Biallelic mutations in either BRCA2 or PALB2 lead to Fanconi anemia and to increased risks of selected childhood cancers, including Wilms tumor.[41,42,43]
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