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

Abstract

Among 225 patients with Wilms'' tumor, 7 had congenital hemihypertrophy (a frequency of 1:32), bringing to 26 the number of cases with this association reported in the literature. In 1 child the cytogenetic study of leukocyte cultures revealed elongation of the long arms of both No. 16 chromosomes; each parent and 2 of 4 siblings had a similar anomaly affecting 1 chromosome of pair 16. Dermatoglyphics on this patient and 3 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 this study by one patient who had a sibling with hemihypertrophy, the 7th 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.