WILMS' TUMOR AND CONGENITAL HEMIHYPERTROPHY: REPORT OF FIVE NEW CASES AND REVIEW OF LITERATURE

Abstract

Among 225 patients with Wilms' tumor seen at the Children's Cancer Research Foundation in Boston, 7 had congenital hemihypertrophy (a frequency of 1:32), bringing to 26 the number of cases reported in the literature with this association. In one child the cytogenetic study of leukocyte cultures revealed elongation of the long arms of both No. 16 chromosomes; each parent and two of four siblings had a similar anomaly affecting one chromosome of pair 16. Dermatoglyphics on this patient and three others in the series were unremarkable, as were studies of urinary gonadotropin excretion. From a review of all cases reported with Wilms' tumor and hemihypertrophy, little was found to indicate a relationship to other disorders, such as Silver's syndrome or neurofibromatosis, in which hemihypertrophy has been described. A role of inheritance was suggested in our series by one patient who had a sibling with hemihypertrophy, the seventh reported familial occurrence of this congenital defect. From the sparse evidence available, it would appear that the origins of hemihypertrophy are heterogeneous and include genic, chromosomal, and other factors which are presently obscure. The association between hemihypertrophy and Wilms' tumor may reflect common etiologic factors or a pre-neoplastic anlage in "hemihypertrophic" kidneys. Since hemihypertrophy seems to be related also to childhood neoplasms originating in the adrenal cortex and liver, further research on this anomaly should enhance our understanding of oncogenic mechanisms.