OBJECTTVE. 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 records 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 retrospectively reviewed by three radiologists. Available pathologic material also was reviewed. RESULTS. Thirty-eight (25%) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n = 19, 13%), caliceal diverticula (n = 2, l%), hydronephrosis (n = 18, 12%), and nephrolithiasis tn = 6,4%). Thirty-three (8’7%) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients, included unnary tract infections (n = 4) and flank pain due to obstructive stone disease (n = 1). Nonmalignant renal disease was mistaken for Wilms’ tumor and nonmalignant renal disease. CONCLUSION. Nonmalignant renal abnormalities occur in approximately 25% of patients with Beckwith-Wiedemann syndrome but are generally asymptomatic. Nonmalignant renal abnormalities should be considered in the differential diagnosis of a mass revealed during screening sonography of a patient with Beckwith-Wiedemann syndrome to avoid unnecessary surgery. Editorial Comment: The authors reviewed the patient records of the Beckwith-Wiedemann registry at the National Cancer Institute. The records included 152 patients 1 to 30 years old (median age 1 year 3 months). Of these patients 38 (25%) actually had 45 nonmalignant renal processes, including medullary cysts (19), caliceal diverticula (2), hydronephrosis (18) and nephrolithiasis (6). Of these patients 33 (87%) were asymptomatic when the abnormality was discovered and the remainder had varying symptoms, including urinary tract infection in 4 and flank pain due to obstructive stone disease in 1. In 2 of these patients nepktomy was performed for suspected Wilms tumor when in fact the patients had nonmalignant renal disease. The most important message in this study is the recognition that not all renal pathology in patients with the Beckwith-Wiedemann syndrome is secondary to Wilms tumors, and that adequate evaluation clinically and radiographically is important before surgery is performed.
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