Hepatoblastoma Biology Study and Tissue Bank

COG 9346 - Lay Summary

Hepatoblastoma Biology Study and Tissue Bank

Although tremendous improvement in the treatment of childhood cancer has resulted from the use of clinical trials, it is clear that additional significant progress will require a better understanding of the molecular makeup of pediatric malignancies. Hepatoblastoma, an embryonal tumor of the liver, is rare in the general population. It occurs in approximately 1 in 50,000 children. However, families affected with Familial Adenomatous Polyposis, in which there is a high incidence of colon cancer, have an 850 fold increase in the frequency of Hepatoblastoma. Hepatoblastoma also occurs in association with Beckwith-Wiedemann Syndrome. Thus the occurrence of Hepatoblastoma suggests the need for both careful investigations into the family history of malignancies in relatives, as well as a search for genetic alterations that may contribute to tumor development.

COG cancer researchers are trying to better understand the causes of Hepatoblastoma. They want to develop improved methods for treatment, diagnosis and possibly even prevention. The exact molecules or genes to be studied will change over time as progress in cancer research is made. Not every gene or molecule known to be involved in cancer will be studied. Researchers will choose to study only those genes or molecules which they think are most promising. At present, the study of at least three genes is planned.

In some cases these changes may be detected in normal tissues such as liver or blood cells. One of the genes to be studied is known as the APC gene. It is linked with colon cancer in adults. Rarely this gene can be passed on in families which are prone to develop both colon cancer and Hepatoblastoma. We don't know how common changes in this gene are in children with Hepatoblastoma. This study will try and figure out if this gene is commonly changed in Hepatoblastoma.