Optic Nerve Glioma

What is an optic nerve glioma?

An optic nerve glioma (also called an optic pathway glioma) is a slow-growing brain tumor that arises in or around the optic nerve, which connects the eye to the brain. As the tumor progresses, it presses on the optic nerve, causing a child’s vision to worsen. Blindness may occur only in about 5 percent of cases. While these are serious tumors, they have a high cure rate.

Nearly 75 percent of optic nerve gliomas, which may affect one or both eyes, occur in children younger than 10, with most younger than 5 years of age at the time of diagnosis. Optic nerve gliomas account for 5 percent of all childhood brain tumors. Because the optic system is located near the hormone center of the brain, these tumors can affect the body's endocrine functions, such as hormone production, salt and water balance, appetite, and sleep.

How we care for optic nerve glioma

Children with optic nerve gliomas are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Glioma Program – one of the world’s largest pediatric glioma treatment programs. Our internationally-recognized pediatric brain tumor specialists have extensive expertise in treating all types of gliomas, including optic nerve gliomas. Our glioma specialists – a team of neuro-oncologists, surgeons, pathologists, and radiation oncologists – focus solely on the care of children diagnosed with gliomas. Our program also offers families the chance to have their child's tumor molecularly profiled (as long as a biopsy can be taken), which may help identify opportunities for targeted treatment.

Our areas of research for optic nerve gliomas

Scientists at both Dana-Farber Cancer Institute and Boston Children’s Hospital are conducting numerous research studies to better clinicians' understanding and treatment of brain tumors and gliomas. Our research program offers patients unparalleled access to clinical trials in which children can receive the newest treatments. We are consistently one of the most well funded pediatric brain tumor centers in the United States, and we pride ourselves on rapidly bringing our discoveries to the aid of our patients.

Dana-Farber/Boston Children's houses the Pediatric Low-Grade Astrocytoma (PLGA) Program, the world's only multidisciplinary clinical and research program dedicated to pediatric low-grade gliomas. Established in 2007 with support from the PLGA Foundation, the program takes a multifaceted approach to finding more effective, less toxic treatments and a cure for children battling brain tumors, and has become the standard bearer for the research and care of pediatric brain tumors. Our pediatric neuro-oncologists, including Pratiti (Mimi) Bandopadhayay, MBBS, PhD, are actively contributing to these efforts. Our program has contributed to international research efforts that have identified genomic drivers that contribute to the growth of pilocytic astrocytoma. Specifically, we have identified genes that are commonly mutated in pilocytic astrocytoma. These findings are guiding clinical trials examining the activity of new drugs specifically for children with optic nerve glioma.

Clinical Trials

Clinical trials, or research studies evaluating new treatment approaches, are a major offering at Dana-Farber/Boston Children’s. Clinical trials are very important for children with hard-to-treat or relapsed conditions.

It’s possible that your child will be eligible to participate in one of the Glioma Program’s current brain tumor clinical trials. In addition to launching our own clinical trials, we also offer trials available through collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-Oncology Consortium (PNOC). We are also the New England Phase I Center of the Children's Oncology Group. If your child has a progressive or recurrent tumor, they may be eligible for a number of experimental therapies available through these groups or from one of our independent clinical investigators.