NONMALIGNANT RENAL-DISEASE IN PEDIATRIC-PATIENTS WITH BECKWITH-WIEDEMANN SYNDROME

OBJECTIVE, The objective of this retrospective review was to determine the incidence and spectrum of nonmalignant renal disease in patients with Beckwith-Wiedemann syndrome. MATERIALS AND METHODS. Patient recor ds were obtained from the Beckwith-Wiedemann Registry of the National Cancer Institute. Imaging findings and medical records of 152 neonates , infants, children, and adults with Beckwith-Wiedemann syndrome (age range, 1 day to 30 years old; median age, 1 year 3 months old) were re trospectively reviewed by three radiologists. Available pathologic mat erial also was reviewed. RESULTS. Thirty-eight (25%) of 152 patients w ith Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalitie s, including medullary renal cysts (n = 19, 13%), caliceal diverticula (n = 2, 1%), hydronephrosis (n = 18, 12%), and nephrolithiasis (a = 6 , 4%). Thirty three (87%) of the 38 patients with nonmalignant renal d isease were asymptomatic. Clinical manifestations of the remaining fiv e patients included urinary tract infections (n = 4) and flank pain du e to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms' tumor in two patients, resulting in unnecessary n ephrectomies. Seven children (18%) had Wilms' tumor and nonmalignant r enal disease. CONCLUSION. Nonmalignant renal abnormalities occur in ap proximately 25% of patients with Beckwith-Wiedemann syndrome but are g enerally asymptomatic. Nonmalignant renal abnormalities should be cons idered in the differential diagnosis of a mass revealed during screeni ng sonography of a patient with Beckwith-Wiedemann syndrome to avoid u nnecessary surgery.