Case 7.11

4-year-old girl with vomiting, diarrhea, fever, and a palpable abdominal mass Axial fat-suppressed FSE (Figure 7.11.1) and axial postgadolinium 3D SPGR (Figure 7.11.2) images show a large heterogeneous mass originating from the anterior right kidney. Wilms tumor Wilms tumor is the most common abdominal malignancy in children, with an annual incidence of 8 cases per 1 million persons. Most patients (80%) present between ages 1 and 5 years, usually with an incidentally discovered abdominal mass. Bilateral tumors are found in 5% of cases, and these are associated with an earlier presentation, a higher incidence of associated congenital abnormalities, and a higher incidence of nephroblastomatosis (multiple rests of persistent embryonic renal parenchyma). Among children with Wilms tumor, 12% have associated congenital abnormalities, including GU abnormalities (5%), hemihypertrophy (2%), and aniridia (1%). Syndromes associated with Wilms tumor include Beckwith-Wiedemann (macroglossia, exomphalos, and gigantism), Denys-Drash (male pseudohermaphroditism and glomerular disease), WAGR (Wilms tumor, aniridia, GU abnormalities, and mental retardation), Sotos (cerebral gigantism), and Bloom (immunodeficiency and facial telangiectasia)....