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Optic Pathway Glioma Treatment Current Page

Optic Pathway Glioma Treatment

Also called: optic nerve tumors, chiasmatic glioma, optic pathway glioma, optic glioma, OPG, low grade glioma, pilocytic astrocytoma

An optic pathway glioma is an old term to describe a low-grade glioma that develops in the parts of the brain that help a person see (the visual system). Most often these tumors belong to a low-grade glioma category called pilocytic astrocytoma. While the name “optic pathway glioma” has been replaced in official medical literature, doctors, patients, and families still use this name.

More than 75% (3 in 4) of optic pathway gliomas occur during the first 10 years of life. Most are diagnosed in children aged 5 or younger. Children who also have the genetic disorder neurofibromatosis type 1 (NF1) are at higher risk for optic pathway gliomas.

Low-grade glioma is the most common central nervous system (CNS) tumor in children. These tumors make up 30% of all childhood CNS tumors.

Studies have shown that almost all low-grade gliomas have specific molecular mutations, or errors, that occur in the tumor cells. For the most part, these mutations can be targeted with new specialized medicines called targeted therapies. This means that molecular testing must occur on the tumor to get the correct diagnosis and determine the best treatment. But if the tumor occurs in the nerve behind the eye, doctors must carefully judge if the tumor can be safely sampled or should be treated without a biopsy.

Find out more about optic pathway glioma on the Together by St. Jude™ online resource.

Treatment of optic pathway glioma

The medical team often includes an oncologist, surgeon, and radiation oncologist, along with nurses and other care staff. They will discuss the types of treatment and help you decide on the best option for your child. The most common treatments are surgery, targeted therapy, chemotherapy, and radiation therapy.

The St. Jude care team will study your child’s case and create a treatment plan for your child. The treatment plan depends on:

Your child’s age
The amount of vision loss
Where the tumor is
If your child has the genetic disorder neurofibromatosis 1 (NF1)

The types of treatment for this tumor are:

Surgery: Surgery can be helpful in optic pathway gliomas for diagnosis and treatment.
- If vision in one eye is very bad, then surgery can completely remove the tumor. This may help cure the disease.
- If all the tumor is removed or very little of the tumor remains after surgery, your child may be closely followed without more treatment and have follow-up MRIs to watch the tumor and see if it grows again.
- But most optic pathway tumors are not completely removed. In these cases, no surgery or a biopsy occurs and more therapy is generally needed.
Targeted therapies are radically changing the way that we treat optic pathway gliomas that cannot be completely removed. New medicines that target tumor cells have resulted in shrinkage of tumors with fewer side effects than traditional chemotherapy or radiation therapy. So, these targeted therapies are becoming our first-choice therapy in this disease.

In optic pathway gliomas, the BRAF gene (DNA) in the tumor cells is commonly altered. This alteration comes in 2 ways: One is known as a BRAF fusion, and the other is a BRAF V600E mutation. Targeted therapy can block the effects of this change.

Chemotherapy uses medicines that stop tumor and cancer cells from growing.
Radiation therapy uses high-energy x-rays or other types of radiation, such as proton therapy, that kill cancer cells or stop them from growing.

Optic pathway glioma clinical trials

St. Jude clinical trials have produced new, groundbreaking treatments for serious childhood diseases. Our goal is to give each child the best treatment with the lowest number of side effects.

Our doctors and scientists work together to find better ways to treat patients. In many cases, new treatments are being developed right here on the St. Jude campus.

We also take part in trials developed by the Children’s Oncology Group (COG) and the Pediatric Brain Tumor Consortium (PBTC). Many St. Jude staff members play key roles in developing these studies. They are large studies carried out at centers across the United States. 

Learn more about clinical research at St. Jude.

Recruiting

HALGG: Hippocampal-Avoidance Using Proton Therapy in Children with Brain Tumors

Study goal:

The main purpose of this study is to find out what happens, good and bad, to memory and learning when the hippocampus is avoided during proton therapy radiation.

Age:

At least 6 years old and younger than 22

Optic pathway glioma care at St. Jude

St. Jude provides the highest quality of care for patients with optic pathway glioma:

We bring together medical experts and specially trained staff to care for your child. We also partner with expert brain surgeons (neurosurgeons) at Le Bonheur Children’s Hospital.
We have a special team that helps understand the makeup of the tumor. This team includes doctors and scientists who study:
- Cancer cells
- How the cancer happened
- Current medicines and treatments to kill cancer cells
- What makes cancer cells grow and what new medicines can stop them
- New medicines and other therapies to improve how treatments work
Our brain and spine tumor clinical trials have led to better therapies in children. These improvements include lower-dose therapies, targeted therapy, and proton therapy. These treatments kill cancer cells while sparing healthy cells. They may lessen side effects that affect brain function after treatment for some types of brain tumors.
Along with the treatment studies constantly being developed at St. Jude, the staff also treats patients on studies developed by the Children’s Oncology Group (COG), the Pediatric Brain Tumor Consortium (PBTC), and the Pacific Pediatric Neuro-Oncology Consortium (PNOC). Many St. Jude staff members play key roles in developing these studies. They are large studies carried out at multiple centers across the United States.

More reasons to choose St. Jude for care include:

We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report.
At St. Jude, we have created an environment where children can be children and families can be together.
We lead more clinical trials for childhood cancer than any other hospital in the U.S. 
St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research to develop new and better approaches to prevent, diagnose, and treat cancer.
The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
Patients may be able to get expert, compassionate care and treatment closer to their homes through our St. Jude Affiliate clinics.

Learn more about brain tumor treatment at St. Jude.

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Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact us

You can start the referral process by letting your physician know you would like a second opinion from St. Jude. They can contact our 24-hour beeper service or contact our Brain Tumor Coordinator. You may also contact the brain tumor coordinator directly with questions.

Learn more about the types of brain tumors we treat at St. Jude and our brain tumor eligibility requirements.