Three cases with co-existent Wilm's tumor and Von Recklinghausen's Disease from a series of 342 nephroblastomas are discussed as to the significance of this association. Biological arguments favor a positive link because of the multiplicity of associative lesions which parallel each other, the co-existence of which suggests a common genetic bond. An embryologic association is described whereby early mutational events, i.e. neural induction of nephrogenesis, may link these two entities in utcro. Further interconnection is stated statistically, in that nephroblastoma patients in this series had a 29 fold higher incidence of multiple neurofibromatosis than predicted for i n the general population. The co-existent diseases in the three cases observed are approximately 3 3 4 0 % more than can be accounted for by chance in the United States population. Malignant transformation in neurofibromatosis is discussed because of the therapeutic implications presented by two of the three Wilms' tumor patients studied.
Cancer 39:2550-2555, 1977.
N EPHROBLASTOMAS (WILMS' TUMOR, EMBRY- oma) account for about 20% of all childhood solid tumors, and approximately 6% of all renal neoplasms, regardless of age at diagnosis. Wilms' tumor is usually diagnosed before the age of five years," is bilateral in 5-15% of the cases, l5 occasionally congenital, l1 and, rarely, familial, 1'~25 suggesting inheritance through a single dominant gene. We have observed in three patients the coexistence of nephroblastoma with Von Recklinghausen's Disease (VRD), an autosomal dominant disorder, suggesting that the gene disorder for VRD may also have an etiologic role for some Wilms' tumors. Although this relationship has been recorded in one previous report," no data have been published to connect these two entities. The purpose of this paper is to document three cases of co-From the