IMAGE: The Lancet Oncology identified six genes that predispose carriers to develop the brain tumor medulloblastoma. view more
Credit: St. Jude Children’s Research Hospital
Researchers have identified six genes that predispose carriers to develop the brain tumor medulloblastoma and have used the discovery to craft genetic counseling and screening guidelines. The study appears today in the journal The Lancet Oncology.
Medulloblastoma is the most common malignant childhood brain tumor and one of the leading causes of non-accidental death in U.S. children and adolescents. The tumor includes four main molecular subgroups with different clinical and biological characteristics as well as treatment outcomes. Except in rare cases associated with genetic disorders like Li-Fraumeni syndrome or Gorlin syndrome, medulloblastoma was thought to occur sporadically by chance, usually in infants and children less than 16 years old.
But researchers completed apparently the largest analysis yet of genetic predisposition in a pediatric brain tumor and found that germline variations in six genes often play a role. The genes include APC, BRCA2 and TP53, which are also associated with an elevated risk for breast, colon, ovarian and other cancers. The findings led the researchers to develop screening and counseling recommendations for patients based on the medulloblastoma molecular subgroups–WNT, sonic hedgehog
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