Beckwith Wiedemann Children's Assn of NZ (Inc)
Assisting families and individuals affected with BWS
Home      BWS screening protocol. VERY IMPORTANT      page 3
Print this pageAdd to Favorite

The second most common cancer in patients with BWS is hepatoblastoma. Similar to Wilms tumor hepatoblastoma can be identified by abdominal ultrasound. However, an abdominal ultrasound does not view the entire liver. Fortunately there is a marker for hepatoblastoma and this is alpha-fetal protein (AFP). AFP is a protein that is made in the liver of unborn infants. At birth the AFP levels are high  and gradually decline to adult levels by 10 or 11 months of age. However, most infants with hepatoblastoma do not have a declining AFP measurement, rather their AFP level increases rapidly.

 

We recently published a case series of 5 patients with BWS who were identified with early stage hepatoblastoma (stage 1) because their AFP level was elevated after serial evaluation of a maximum of 8 weeks.-J Pediatr. 2003 Aug; 143(2):270-2. A concerning AFP level is one that increases dramatically and one that does not continuing drop during the first year of life. For example, an AFP increasing from 18 to 180 ng/cc would warrant further investigation or an AFP level that did not decline when an infant was 6 months of age. When such situations occur, we recommend that the patient have a repeat AFP measurement in approximately two weeks with possibly an imaging study (liver ultrasound, CT scan or MRI scan). Several children had elevated AFP measurements where the imaging studies did not initially real the tumor.

 

Hepatoblastoma is also a fast growing cancer and because of the fast growth, we recommend an AFP measurement every 6 weeks and ultrasound of the liver and kidney every 12 weeks. The ultrasound of the liver can be done at the same time as the ultrasound of kidney. Unlike Wilms tumor, the risk of hepatoblastoma drops off after 4 years, so we would recommend screening with AFP up to 4 years of age. We don't see any value in conducting a liver ultrasound after four years of age.

 

Back to Page 2                                                                                                                                                     Continue to Page 4

 

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