Study searches for families with glioma

In the United States, 23,720 new cases of glioma are diagnosed every year, but little is known about how or why they occur despite decades of research and technological advancement. Rarer still is a little-known population of families for whom the most aggressive form of brain cancer has struck two or more times. Gliogene, the first study to research brain tumor susceptibility in families, is looking for the estimated 5 to 8 percent of glioma patients who carry this genetic predisposition.

Dr. Melissa Bondy, professor of medicine and epidemiology and population sciences at Baylor College of Medicine and principal investigator of Gliogene, has devoted her 30-year career to the study of familial brain tumors. In 2014, Bondy released the findings from the first phase of the Gliogene study, which sequenced the whole exome of 101 individuals with glioma from 70 families. The study uncovered the first gene thought to be associated with familial glioma, called POT1. More patients are needed for the study to identify new familial links and make any further scientific breakthroughs.

“Simply put, we need to find the needle in the haystack. Because glioma is rare and life expectancy short, it is a tremendous challenge to locate and recruit glioma patients with this susceptibility. We are calling on the neuro-oncology, medical and patient communities to spread the word and help us find these patients,” said Bondy, who also is a McNair Scholar, Dan L Duncan Chair and associate director of Cancer Prevention and Population Sciences in the Dan L Duncan Comprehensive Cancer Center at Baylor. “It is our hope that results from Gliogene will further brain cancer screening and prevention strategies for future generations.”

Forms of glioma, a malignant primary brain tumor, include astrocytoma, oligodendroglioma and glioblastoma multiforme.

“We are starting to think about glioma in the same way that we now approach the BRCA breast cancer genes. We know that BRCA mutations can be inherited and put patients at a higher risk for breast cancer, so we hope we can identify and generate the same kind of awareness for glioma through enrolling and sequencing families from across the country,” Bondy said.

Participants may be eligible to participate in Gliogene if they and a biological family member both have been diagnosed with glioma, even if the family member is deceased, or if two biological family members have been diagnosed.

The mission of Gliogene hits particularly close to home for, sisters who have lost three family members to glioma, including their father, grandmother and an aunt. “Our experience has made us tireless advocates for brain cancer awareness. Gliogene is an important initiative, and the answers that may be uncovered through this study could benefit countless families,” Davis said.

Enrollment in Gliogene is now open. Interested participants can fill out a form with more information, and the study can be completed entirely by phone and mail. For more information about Gliogene and how to participate, visit the study website.

This study is supported by the National Cancer Institute R01CA217105. Dr. Benjamin Deneen, professor in the Stem Cells and Regenerative Medicine Center and member of the Dan L Duncan Comprehensive Cancer Center at Baylor College of Medicine is co-principal investigator on this study, and Dr. Matthew Bainbridge, associate director of clinical genomics research at the Genomics Institute at Rady Children’s Hospital, is co-principal investigator of Gliogene.