PEDIATRICS Vol. 81 No. 1 January 1988, pp. 147-149
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Heritable Fraction of Unilateral Wilms Tumor

Frederick P. Li MD1, Wick R. Williams PhD1, Kathreen Gimbrere BA1, Francoise Flamant MD1, Daniel M. Green MD1, and Anna T. Meadows MD1

1 From the Clinical Studies Section, National Cancer Institute, Bethesda, Maryland; Fox Chase Cancer Center, Philadelphia; Dana-Farber Cancer Institute, Boston; Institute Gustave Roussy, Villejuif, France; Roswell Park Memorial Institute, Buffalo, New York; and Children's Hospital of Philadelphia

Heritability of the unilateral-sporadic (non-familial) form of Wilms tumor was examined in the offspring of 96 long-term survivors of the neoplasm. No Wilms tumor has developed in any of the 179 offspring of these patients. The maximum likelihood estimate of a hereditary Wilms tumor in our patients is zero and the corresponding 95% upper confidence limit ranges between 0.06 and 0.11, depending on penetrance. Among their offspring, the 95% upper bound of the risk of Wilms tumor is 0.02. These figures can be applied in genetic counseling of other survivors of unilateral-sporadic Wilms tumor.

Key Words: Wilms tumor • cancer genetics • counseling

Submitted on December 18, 1986
Accepted on April 13, 1987




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