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Genetic cause identified in rare pediatric brain tumor

  • Keith Ligon, MD, PhD

    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. Named for their curious behavior of “hugging” blood vessels in the brain, angiocentric gliomas are usually cured with surgery and don’t need further treatment with radiation or chemotherapy. But given their recent description and difficulties in identifying them confidently, some patients receive the additional therapy, which often is damaging to the growing brain, in an effort to prevent a recurrence.

    “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, a senior author of the report in Nature Genetics along with Keith Ligon, MD, PhD, and Adam Resnick, PhD. Ligon is a pathologist at Dana-Farber/Boston Children’s Hospital Cancer and Blood Disorders Center and Dana-Farber/Brigham and Women’s Cancer Center (DF/BWCC). Resnick is an investigator at Children’s Hospital of Philadelphia.

    Angiocentric gliomas were recognized as distinct entities less than 10 years ago. Their name reflects the tumor cells’ tendency to line up around blood vessels. Fewer than 30 cases have been described in medical literature. Seizures are typically the first symptom leading to diagnosis. Although they are classified as a tumor, they are not malignant and don’t spread to other parts of the body.

Posted on February 01, 2016

  • Keith L. Ligon, MD, PhD
  • Research
  • Rare Tumors, Childhood
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