Correlation of chromosome abnormalities with histological and clinical features in Wilms' and other childhood renal tumors.

Chromosomes and histology were successfully studied in 33 childhood renal tumors. Thirty-one tumors were classified as one of four subtypes of Wilms' tumor. Of 24 typical Wilms' tumors, 12 had hyperdiploidy with nonrandom trisomies, mostly including +6 and/or +12. Three typical Wilms' tumors with an 11p13 deletion or a pericentric inversion with a break in 11p13 were not associated with aniridia. Two other typical Wilms' tumors with the 11p13 deletion and one fetal rhabdomyomatous nephroblastoma with an 11p13 translocation were associated with aniridia. Two cystic partially differentiated nephroblastomas showed hyperdiploidy with +12. Of four clear cell sarcomas of the kidney, three had normal diploidy and the other had a 2;22 translocation. Two congenital mesoblastic nephromas had hyperdiploid karyotype with trisomy 11, which was never seen in the 31 Wilms' tumors. Our findings and a review of data on 102 reported Wilms' tumors revealed 11p13 abnormalities in 24 tumors, 11p15 abnormalities in five tumors, and partial deletions of 1p, 7p, 11q, 12q, 16q, or 17p or monosomy of No. 21 or No. 22 each in four or more tumors. These findings suggest that increased copy number of genes on the nonrandom trisomic chromosomes might contribute to the genesis of many Wilm's tumors and that deletion of various tumor suppressor genes other than a Wilms' tumor gene, WT1 in 11p13, might also play a critical role in the development of some tumors.