2-year-old child is seen for a well-child visit in a pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea where the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month well-child visit. Physical examination is otherwise unremarkable. Abnormalities of which of the following chromosomes have been linked to this disease?
Inactivation of the WT-1 Wilms tumor gene (located at 11p13, and thought to encode a DNA-binding protein impoant in fetal kidney development) has been repoed in the tumor cells from many Wilms tumor cases. Also, the WAGR syndrome involves a deletion of chromosome 11p13 and the Beckwith-Wiedemann syndrome involves a rearrangement of chromosome 11p15. The genetic basis of the Drash syndrome has not been established. The other choices are distractors. Ref: Konety B.R., Williams R.D. (2008). Chapter 21. Renal Parenchymal Neoplasms. In E.A. Tanagho, J.W. McAninch (Eds), Smith's General Urology, 17e.
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