Brain CancerA Parent’s Guide to pLGG Management

What Is Pediatric Low-Grade Glioma?

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Updated on February 19, 2025
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Pediatric low-grade gliomas are a type of slow-growing tumor of the brain and spinal cord in children.

 These tumors are less likely to spread to surrounding tissues than higher-grade tumors, so they often have better outcomes.

Types of Pediatric Low-Grade Glioma

Gliomas aren’t one specific type of cancer — rather, they can be a number of different tumors.

 In the past, pediatric gliomas were classified into low-grade and high-grade gliomas based on the cells and tissues they originated in, but in 2021 were classified into subtypes by the World Health Organization based on additional features, including how they spread in the body.

There are three types of pediatric low-grade gliomas:

  • Pediatric-type low-grade gliomas
  • Circumscribed astrocytic gliomas
  • Glioneuronal and neuronal tumors

Types of Pediatric-Type Diffuse Low-Grade Gliomas

These tumors usually occur in the cerebrum, which is the largest part of the brain, but they can also occur in the cerebellum, located at the back of the head. Rarely, they occur in the optic pathway (such as the optic nerve) and hypothalamus. They’re referred to as “diffuse” tumors because they don’t have a clear border when they’re viewed under a microscope. Pediatric type diffuse low-grade gliomas include:

  • Diffuse low-grade glioma, MAPK pathway-altered: These tumors usually have an alteration in the BRAF gene located on the MAPK pathway.
  • Diffuse astrocytoma, MYB- or MYBL1-altered: These tumors have MYB gene alterations.

Types of Circumscribed Astrocytic Gliomas

These tumors can occur in the cerebellum, brain stem, and optic pathway, among other areas of the central nervous system. In contrast to diffuse tumors, circumscribed gliomas have a clearer border. They include:

  • Pilocytic astrocytoma: These tumors can occur in the cerebellum, brain stem, optic pathway, and hypothalamus (the area of the brain that produces hormones).
  • Subependymal giant cell astrocytomas (SEGAs): These grade 1 tumors occur in the brain’s ventricles (cavities). SEGAs are almost always linked with a genetic condition called tuberous sclerosis, which is caused by changes in either the TSC1 or TSC2 gene.

  • Pleomorphic xanthoastrocytomas (PXAs): These grade 2 tumors usually occur in the brain.

Types of Glioneuronal and Neuronal Tumors

These tumors occur in the nerve cells; glioneuronal tumors are composed of both neuronal and glial cells (located in the nervous system) and neuronal tumors are composed of only neuronal cells. Rarely, they spread to the nearby tissue.

  • Ganglioglioma: These tumors typically arise in the cerebral cortex and are associated with seizures.
  • Desmoplastic infantile ganglioglioma/desmoplastic infantile astrocytoma: These tumors often appear within the first year of life; they can have a genetic alteration in the BRAF gene.
  • Dysembryoplastic neuroepithelial tumor: These tumors can appear in mid- to late adolescence, usually in the temporal lobe.

Signs and Symptoms of Pediatric Low-Grade Gliomas

Illustrative graphic titled Symptoms of Pediatric Low-Grade Glioma shows Headaches, Severe or frequent vomiting, Vision problems, Premature puberty, Trouble walking or balancing, Seizures, Weight gain or weight loss, Confusion, Changes in behavior
Everyday Health
The symptoms of childhood glioma occur when the tumor presses on surrounding areas of the brain. Some of the most common signs include:

  • Headaches (especially if they occur in the morning or get better after vomiting)
  • Severe or frequent vomiting (without other signs of gastrointestinal upset)
  • Vision problems, such as double vision, blurry vision, or vision loss
  • Premature puberty
  • Trouble walking or balancing
  • Seizures
  • Weight gain or weight loss
  • Confusion
  • Changes in behavior
  • Sleepiness

Causes and Risk Factors of Pediatric Low-Grade Gliomas

There aren’t many risk factors for pediatric brain and spinal cord cancers. Lifestyle factors, such as body weight and tobacco use, often play a role in adult cancers, but because those factors take years to make an impact, they usually don’t influence childhood cancers.

Radiation exposure can increase the risk for brain cancer, but radiation-induced tumors are fairly rare. When they do occur, they usually develop around 10 to 15 years after radiation therapy (which may be given to treat other cancers, such as leukemia).

In rare cases, an inherited gene condition can increase the risk of certain pediatric low-grade gliomas. These conditions include:

  • Neurofibromatosis type 1 (von Recklinghausen disease): Has been linked to optic pathway gliomas and is thought to be caused by changes in the NF1 gene
  • Neurofibromatosis type 2: Less common than type 1, but has been linked to spinal cord gliomas and is caused by changes in the NF2 gene
  • Tuberous sclerosis: An inherited condition that is linked to SEGAs, which may be caused by changes in either the TSC1 or TSC2 gene

How Are Pediatric Low-Grade Gliomas Diagnosed?

If your child is showing signs or experiencing symptoms of a tumor, the following tests may be done to confirm a diagnosis.

  • Medical history and physical exam: May test reflexes, muscle strength, vision and eye movements, and coordination, among other functions
  • Magnetic resonance imaging (MRI) test: Uses a strong magnet (rather than radiation) to take images of the brain or spinal cord to detect the presence of a tumor. An MRI is the best way to find tumors in the brain and spinal cord.
  • CT scan: Uses X-rays to make detailed images of the brain and spinal cord (MRIs are more commonly used)
  • PET scan: Involves injecting a radioactive substance into the blood and using a special camera to create a picture of areas of radioactivity in the body. A PET scan is most helpful for detecting fast-growing tumors.
  • Lumbar puncture (spinal tap): A test that looks for signs of cancer in the cerebrospinal fluid (the fluid in the brain and spinal cord). During a spinal tap, a doctor injects a needle between the bones of the spine to withdraw fluid, which is then examined for cancer cells.
  • Biopsy: A sample of the tumor is removed to be examined for cancer cells.

Treatment and Medication Options for Pediatric Low-Grade Gliomas

The treatment for pediatric low-grade gliomas depends on the type of tumor and where it’s located. The most common treatments are surgery, chemotherapy, and targeted therapy. (Radiation therapy is usually used for high-grade gliomas only.)

Surgery

Surgery is often the first treatment for children with a low-grade glioma. The goal is to remove all of the tumor or as much of it as possible. Doing so can control or cure the tumor, as well as help ease some of the symptoms.

The most common surgery for brain tumors is called a craniotomy. During the procedure, an opening is made in the skull to remove the tumor, either with a scalpel, special scissors, or a suction device. Afterward, the piece of the skull is replaced and the incision is closed.

Some tumors — such as those that are located deep within the brain or in the brain stem — can’t be removed with surgery. In that case, other treatments would be necessary.

Chemotherapy

Chemotherapy (chemo) is the use of cancer-fighting drugs to shrink or destroy the tumor. They’re usually given intravenously or in pill form. Chemo may be used alongside surgery.

For children younger than 3 years, chemotherapy is sometimes used to slow a tumor’s growth if it can’t be completely removed with surgery — or if it returns. When your child is older, they may be treated with radiation.

Targeted Therapy Drugs

Targeted drugs are treatments for brain and spinal cord tumors in children, but they can be useful in certain cases.

For example, in the tumor cells of some low-grade gliomas, there are mutations in the BRAF gene that cause them to make certain proteins, which help them grow. The targeted therapy drugs dabrafenib (Tafinlar) and tovorafenib (Ojemda) target this BRAF gene, while the drug trametinib (Mekinist) targets a related MEK protein. Sometimes, these drugs are used in combination with one another for the best possible outcome.

Other targeted therapy drugs include vorasidenib (Voranigo), which blocks IDH1 and IDH2 proteins to help the tumor cells mature into more normal cells, and everolimus (Afinitor), which blocks a protein called mTOR that usually helps cells grow and divide.

Prevention of Pediatric Low-Grade Gliomas

It’s not clear whether pediatric low-grade gliomas can be prevented. Other than exposure to radiation therapy, there aren’t any known lifestyle-related risk factors for brain and spinal cord tumors.

Pediatric Low-Grade Gliomas Prognosis

Pediatric low-grade gliomas often have a favorable prognosis. The 10-year survival rate for pilocytic astrocytoma is 96 percent, while other low-grade gliomas have an 85 percent survival rate.

It’s uncommon for pediatric low-grade gliomas to continue into adulthood.

Research and Statistics

Pediatric low-grade gliomas are the most common type of brain and spinal tumors in children, accounting for about 1 in every 3 pediatric tumors of the brain and spinal cord.

Support for People With Pediatric Low-Grade Gliomas

A tumor diagnosis is stressful for you and your child. There are support groups that can help you along your journey. Consider joining a group offered by one of the following organizations.

Pediatric Brain Tumor Foundation

This national nonprofit organization offers one-on-one support for children with cancer, their siblings, and other family members, as well as virtual support groups for parents and caregivers.

Children’s Brain Tumor Foundation
This organization guides families through a child’s brain tumor journey, providing support during diagnosis, treatment, and life after cancer.

The Takeaway

  • Pediatric low-grade gliomas are slow-growing tumors that affect the brain and spinal cord in children.
  • Some symptoms of low-grade gliomas include headaches (especially in the morning), vomiting, and vision problems.
  • Low-grade gliomas in children may be treated with surgery, chemotherapy, or targeted drugs.
  • While everyone’s prognosis is different, pediatric low-grade gliomas tend to be treatable and are associated with positive outcomes.

Resources We Trust

EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
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  2. Types of Brain and Spinal Cord Tumors in Children. American Cancer Society. June 20, 2018.
  3. Childhood Astrocytomas, Other Gliomas, and Glioneuronal/Neuronal Tumors Treatment (PDQ) — Health Professional Version. National Cancer Institute. January 6, 2025.
  4. Park YW et al. The 2021 WHO Classification for Gliomas and Implications on Imaging Diagnosis: Part 2—Summary of Imaging Findings on Pediatric‐Type Diffuse High‐Grade Gliomas, Pediatric‐Type Diffuse Low‐Grade Gliomas, and Circumscribed Astrocytic Gliomas. Journal of Magnetic Resonance Imaging. April 17, 2023.
  5. Risk Factors for Brain and Spinal Cord Tumors in Children. American Cancer Society. June 20, 2018.
  6. Glioneuronal and Neuronal. American Brain Tumor Association. March 2023.
  7. Park YW et al. The 2021 WHO Classification for Gliomas and Implications on Imaging Diagnosis: Part 3 — Summary of Imaging Findings on Glioneuronal and Neuronal Tumors. Journal of Magnetic Resonance Imaging. September 16, 2023.
  8. Childhood Glioma Program. Dana-Farber Cancer Institute.
  9. Tests for Brain and Spinal Cord Tumors in Children. American Cancer Society. August 8, 2024.
  10. Childhood Low-Grade Gliomas. Dana-Farber Cancer Institute.
  11. Surgery for Brain and Spinal Cord Tumors in Children. American Cancer Society. June 20, 2018.
  12. Chemotherapy for Brain and Spinal Cord Tumors in Children. American Cancer Society. June 20, 2018.
  13. Targeted Therapy Drugs for Brain and Spinal Cord Tumors in Children. American Cancer Society. August 8, 2024.
  14. Can Brain and Spinal Cord Tumors in Children Be Prevented? American Cancer Society. June 20, 2018.
  15. Manoharan N et al. Pediatric Low-Grade Glioma: Targeted Therapeutics and Clinical Trials in the Molecular Era. Neoplasia. February 2023.
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Walter Tsang, MD

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Walter Tsang, MD, is a board-certified medical oncologist, hematologist, and lifestyle medicine specialist. Inspired by the ancient Eastern philosophy of yang sheng ("nourishing life"), Dr. Tsang has developed a unique whole-person oncology approach that tailors cancer care and lifestyle recommendations to each patients’ biopsychosocial-spiritual circumstances. He partners with patients on their cancer journeys, emphasizing empowerment, prevention, holistic wellness, quality of life, supportive care, and realistic goals and expectations. This practice model improves clinical outcomes and reduces costs for both patients and the healthcare system. 

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