AN UNUSUAL CYSTIC LESION HISTOLOGICALLY SIMILAR TO AUTOSOMAL-DOMINANTPOLYCYSTIC KIDNEY-DISEASE IN A CHILD WITH ANIRIDIA AND DEL 11P13

The association of Wilms tumor with an interstitial deletion on the sh ort arm of chromosome 11 is well established. Specifically, the 11p13 band has been implicated in the syndrome of Wilms tumor, aniridia, gen itourinary abnormalities and mental retardation. Only rarely have othe r renal lesions been associated with the chromosomal abnormality del 1 1p13. We report a case of a segmental cystic lesion, histologically ak in to autosomal dominant polycystic kidney disease, in a child with an iridia and the del (11)(p12p14) karyotype.