Beckwith Wiedemann Children's Assn of NZ (Inc)
Assisting families and individuals affected with BWS
Home      BWS screening protocol. VERY IMPORTANT
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Ongoing Screening Protocol (in brief)
 
1. AFP blood test every 6 weeks from birth to 4 years old
2. Kidney and Liver ultrasound every 3 months from birth to 8 years
 
Screening Protocol for Beckwith Wiedemann Syndrome
MICHAEL R. DEBAUN, MD. MPH
PRINCIPAL INVESTIGATOR FOR BWS REGISTRY
 

We recommend screening for cancer in children with BWS, despite the observation that most children with BWS do not develop cancer. Approximately 1 of 10 children with BWS develops cancer, however the risk is high enough to warrant screening for cancer. The incidence of cancer is age dependent with the risk being higher in the first four years, lower between 5 and 10 years and near the baseline risk of cancer in the general population by 10 years of age. J Pediatr 1998:132:398-400.

 

The major reason to screen for cancer is because early identification of the cancer leads to improved survival and/or decreased morbidity associated with cancer treatment. Fortunately, the two most common cancers in BWS, namely Wilms tumor and hepatoblastoma, meet these criteria.

 

Wilms tumor is the most common cancer in children with BWS, occurring in about 5-7% of all children with BWS. Most children develop Wilms tumor prior to their fourth birthday; however children with BWS can develop Wilms tumor up to 7 or 8 years of age 95% of all Wilms tumor have occurred. J Pediatr 1998 Mar; 132 (3 Pt 1): 377-9.

 

The length of screening interval for ultrasound examination is not well established, however we believe screening in the intervals less than four months is appropriate. In one of our studies we demonstrated that screening with ultrasound with Wilms tumor in intervals of a least 4 months resulted all of the patients having early stage Wilms tumor, whereas patients who were not screened had a much lower frequency of late stage disease.

 

Two common but false statements are often mentioned about screening for Wilms tumor. First is that screening with the parents hand on the abdomen is just as effective as an ultrasound examination. This is simply not true. In the patients that we identified by ultrasound screening the average size of the tumor was 4cms as opposed to 12cms when the Wilms tumor is identified. The ultrasound is simply a better way to identify Wilms tumor when compared to abdominal palpation. The second false perception is the screening interval can be increased from 3 months to 6 months to 12 months as a child becomes older. This is simply not true because Wilms tumor grows too fast to justify screening every 6 months to 12 months. In fact, we have several patients who were screened every 6 months who had late stage Wilms tumor.