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Familial Wilms' tumor: A descriptive study

✍ Scribed by Breslow, Norman; Olson, Jane; Moksness, Jami; Beckwith, J. Bruce; Grundy, Paul

Book ID: 102647347
Publisher: John Wiley and Sons
Year: 1996
Tongue: English
Weight: 596 KB
Volume: 27
Category: Article
ISSN: 0098-1532
DOI: 10.1002/(sici)1096-911x(199611)27:5<398::aid-mpo2>3.0.co;2-h

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✦ Synopsis

Among 6,209 patients with Wilms' tumor entered o n the National Wilms' Tumor Study (NWTS), 93 patients (1.5%) from 63 families had a positive family history. In 30 of these 63 families a (half) sibling or parent of the NWTS patient was confirmed to have had Wilms' tumor. Fifteen (16.1%) of the familial, but only 7.1 % o f sporadic cases, had bilateral disease.

Mean ages at diagnosis were 15.8 vs. 35.2 months ( P = 0.012) for bilateral vs. unilateral familial cases and 32.0 vs. 44.7 months for sporadic cases. lntralobar nephrogenic rests were found twice as frequently in association with the tumors of familial as with those of sporadic cases. Cases of bilateral and metastatic disease tended to cluster within specific families, suggesting heterogeneity in the genetic etiology. The number and age distribution of familial cases transmitted through the father were about the same as those of cases transmitted through the mother. This finding is inconsistent with models of genomic imprinting that involve fam i I ial transmission of a tumor-suppressor gene and it casts further doubt on the hypothesis that all bilateral cases are hereditary.

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