Beckwith Wiedemann Children's Assn of NZ (Inc)
Assisting families and individuals affected with BWS
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Once a child has been diagnosed with Wilms tumor, we would strongly encourage an evaluation for a partial nephrectomy. We have recently demonstrated that children with BWS who developed cancer and were screen in intervals of less than four months had an average Wilms tumor size of approximately 3.5 cm, as opposed to 11 to 13 cm in children with Wilms tumor that are not screened, yet none of the patients received a partial nephrectomy and most would have been considered reasonable candidates for a partial nephrectomy - J Pediatr Surg. 2002 Jan;37 (1):57-60.

 

We believe consideration for a partial nephrectomy is important because of the risk of non malignant renal disease and metachronous Wilms tumor. 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. Thirty-eight (25%) of 152 patients with Beckwith-Wiedemann syndrome had 45 nonmalignant renal abnormalities, including medullary renal cysts (n=19, 13%), coliceal diverticula (n=2.1%), hydronephrosis (n=18, 12%), and nephrolithiosis (n=6.4%). Thirty-three (87%) of the 38 patients with nonmalignant renal disease were asymptomatic. Clinical manifestations of the remaining five patients included urinary tract infections (n=4) and flank pain due to obstructive stone disease (n=1). Nonmalignant  renal disease was mistaken for Wilms tumor in two patients, resulting in unnecessary nephrectomies. Seven children (18%) had Wilms tumor and nonmalignant renal disease..Am J Radiology 1998; 171:733-737

 

We recently introduced a protocol to the Children's Oncology Group that awaiting approval for conducting the partial nephrectomy trial in children with BWS and Wilms Tumor (2003). We are awaiting approval from this trial.
 
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A full copy of this Screening Protocol can be downloaded for printing by clicking here

A chart of expected AFP (Alpha Feto-Protein) Levels for children with Beckwith-Wiedemann Syndrome can be downloaded here