Beckwith Wiedemann Children's Assn of NZ (Inc)
Assisting families and individuals affected with BWS
Home      BWS screening protocol. VERY IMPORTANT      page 4
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As with all screening programs there is a risk benefit ratio that has to be determined by each parent and physician for the child with BWS. Our recommendations to screen with AFP up to age 4 years and ultrasound up to 8 years is based on the observation that most but not all hepatoblastoma and Wilms tumor will occur by these ages. The decision to screen beyond these ages is an individual one, where the benefits must be weighed against the risks. The major risk of screening is a misdiagnosis of cancer resulting in inappropriate surgery. We have had three such incidents. We conducted a cost effective model describing the costs and benefits of screening for cancer in this population; although not perfect, the model coupled with the data strongly favors screening for Wilms tumor and hepatoblastoma based on the above mentioned data. Med Pediatr Oncol. 2001 Oct;37(4):349-56.

 

Children with BWS can develop other cancers like neuroblastoma, rhabdomyosarcoma, or adrenocortical carcinoma; however, there is no proven benefit in screening for these cancers. Fortunately they are rare in children with BWS.

 

Please feel free to contact me if you have any questions.

 

Warm regards,

Michael DeBaun, M.D., MPH

Principal Investigator of the BWS Registry

Associate Professor of Pediatrics and Biostatistics
 

Article References for the Screening Protocol for BWS

 

DeBaun MR. Tucker MA: Risk of cancer during the first four years of life in Children from the Beckwith-Wiedemann Syndrome Registry.  J Pediatr. 1998 Mar;132(3 Pt 1):398-400.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9544889)

 

Choyke P. Siegel MJ. Oz O. Sotelo-Avila C, DeBaun MR: Non-malignant renal disease in pediatric patients with Beckwith-Wiedemann syndrome. AM J Radiology 1998; 171:733-737.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9725306)

 

DeBaun MR, Brown M. Kessler L: Screening for Wilms' tumor in children with high-risk congenital syndromes: Considerations for an intervention trial.  Med Pediatr Oncol 1996:27:415-421.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=8827068)

 

Choyke PL. Siegel MJ, Craft AW. Green DM, DeBaun MR: Screening for Wilms tumor in children with Beckwith Wiedemann Syndrome or idiopathic hemihypertrophy. Med Pediatr Oncol 1999; 32:196-200.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10064187)

 

Clericuzio CL, Chen E, McNeil DE, O'Connor T, Zackai EH, Medne L, Tomlinson G, DeBaun M.:Serum alpha-fetoprotein screening for hepatoblastoma in children with Beckwith-Wiedemann syndrome or isolated hemihyperplasia. J Pediatr. 2003 Aug; 143(2):270-2.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=12970646)

 

Lahdenne P, Kuusela P, Siimes MA, Ronnholm KA, Salmenpera L, Heikinheimo M.: Biphasic reduction and concanavalin A binding properties of serum alpha-fetoprotein in preterm and term infants. J Pediatr. 1991 Feb;118(2):272-6.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=1704432)

 

Beckwith JB.: Children at increased risk for Wilms tumor: monitoring issues. J Pediatr. 1998 Mar;132(3 Pt 1):377-9.

(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=9544882)

 

McNeil DE, Brown M, Ching A, DeBaun MR.: Screening for Wilms tumor and hepatoblastoma in children with Beckwith-Wiedemann syndromes: a cost-effective model. Med Pediatr Oncol. 2001 Oct;37(4):349-56.

 
 
 

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