Genetic cause identified in rare Pediatric Brain tumor

Diagnosis and treatment decisions for a recently recognized type of children's brain tumor should be improved by the discovery of the genetic mechanism that causes it, say researchers who identified the unusual DNA abnormality in angiocentric gliomas. Currently there is no definitive pathological test to help identify this rare type of low-grade glioma.
"Now we know these angiocentric gliomas have a different biology, and we have an exact way of identifying them so that patients can avoid this additional therapy that has life-long consequences," said Rameen Beroukhim, MD, PhD, of Dana-Farber Cancer Institute. Because of these findings, the researchers said, angiocentric glioma should be classified as a separate biologic entity, with the presence of the gene fusion confirming the diagnosis. "This could aid in distinguishing angiocentric glioma from tumors with higher potential for recurrence that could require further treatment," they said. The authors have developed the first genetic test now available for these patients through collaboration with cytogeneticist Azra H. Ligon, PhD.