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Low-grade glioma

Also called: Pilocytic Astrocytoma (PA), Pilomyxoid Astrocytoma (PMA), pleomorphic xanthroastrocytoma (PXA), ganglioglioma, gangliocytoma, diffuse astrocytoma, papillary glioneuronal tumor, rosette-forming glioneuronal tumor, diffuse leptomeningeal glioneuronal tumor, central neurocytoma

What is a low-grade glioma?

Gliomas are a type of brain or spine tumor formed from glial cells. Glial cells are non-neuronal cells or non-nerve cells in the central nervous system (CNS). There are four different types of glial cells: astrocytes, oligodendrocytes, microglia and ependymal cells. These function to support and protect the neurons or “nerves” in the brain. They support the nerves by providing structure (like scaffolding) to grow on, supply food and oxygen, and remove dead cells and cellular debris. Rarely, these cells can go wrong and grow into gliomas.

Once diagnosed, a glioma is graded based on how abnormal the cells appear and how quickly they are growing or dividing. Grade I or grade II gliomas are considered low-grade gliomas. These tumors are typically slow growing and less likely to spread (metastasize) but can cause many problems and need medical treatment.

Just as there are different types of glial cells, there are different types of low-grade gliomas. The name of the tumor generally refers to the type of glial cell it originates from. For example, the most common pediatric low-grade glioma is an astrocytoma, which comes from astrocytes.

Diagram of brain

Gliomas can form in several different areas of the brain and spine including:

  • Cerebellum – area that coordinates movement, balance and posture
  • Cerebrum – area that controls thinking, speech, emotions, reading, writing, problem-solving and movement
  • Brainstem – area that controls breathing, heart rate, facial movements, eye movements, swallowing and many other functions
  • Hypothalamus – area that controls body temperature, thirst and hunger
  • Visual pathway – area that controls a group of nerves that connects the eyes to the brain
  • Optic nerve – nerve that controls vision
  • Spinal cord – cluster of nerves and glial cells that connects the nerves of the body with the brain.

How common is low-grade glioma?

Pediatric low-grade gliomas are the most common central nervous system (CNS) tumor in children. They make up 30% of all childhood CNS tumors.

What are the symptoms of a low-grade glioma?

The symptoms related to low-grade gliomas can vary based on the size of the tumor as well as where it is in the brain. The child’s age and how fast the tumor grows can also dictate the symptoms experienced. Symptoms may include:

  • Vision, hearing or speech problems
  • Headaches, especially in the early morning
  • Loss of balance or trouble walking
  • Weakness, tingling or changes of feeling on one side
  • Nausea and vomiting
  • Unexplained weight gain or loss
  • Seizures
  • Changes in personality or behavior
  • Increased head size (in infants)

How is low-grade glioma treated?

  •   Surgery – is used to both treat and diagnose low-grade gliomas.
    • A biopsy is a surgery performed to get a piece of the tumor in order to diagnose the tumor by type and grade.
    • A resection is a surgery performed to get most or all the tumor removed.
    • For many low-grade gliomas, surgery is curative if the tumor is completely removed.
    • If very little tumor remains after surgery, the patient can be closely followed with an MRI.
  • Chemotherapy (“chemo”) – is a medicine given after surgery to kill cancer cells or stop them from growing and dividing. This treatment is given by mouth or as an injection into the blood stream. If more than one type of chemo is given at a time, it is known as combination therapy. Examples of chemotherapy commonly used in low grade gliomas are vincristine, carboplatin, vinblastine and lomustine.

  • Targeted Therapy – is a more specialized kind of chemotherapy that is designed to “target” or attack a specific marker in the tumor. This marker is the change in the cancer cell that makes it unique from healthy cells. This therapy can keep cancer cells from growing or repairing and can tell the cancer cell to destroy itself. Targeted therapy is usually given by mouth.  Examples of targeted therapy for low-grade gliomas are selumetinib, trametinib, mirdametinib and everolimus. 
  • Radiation Therapy – uses high-energy X-rays or other types of radiation, such as proton beam, to kill cancer cells or stop them from growing. The way the radiation is given depends on the type of tumor, disease risk and where the tumor is in the brain or spinal cord.

Your medical team, often made up of an oncologist, surgeon and radiation oncologist, can discuss the types of therapy with you and decide on the best option for your child.

What are the survival rates for low-grade glioma?

Survival rates for low-grade gliomas in children are very good, with 10-year survival rate exceeding 85. Individual results vary widely based on the following factors:

  • The specific type, location and grade of the glioma
  • If the tumor has been totally resected
  • If the tumor is localized to one area or has spread
  • The age of the child when diagnosed
  • Whether the tumor is newly diagnosed or has returned (recurred)

Why choose St. Jude for your child’s low-grade glioma treatment?

  • St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
  • St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
  • The St. Jude Brain Tumor Program is one of the largest in the country filled with expert doctors from many medical fields. These include pediatric neurosurgeons from Le Bonheur Children’s Hospital who operate on the brain, skull and spinal cord; pediatric neuro-oncologists who study and treat cancer in the brain and nervous system; and radiation oncologists, who are experienced in using state of the art proton beam radiation therapy. This diverse knowledge is vital to the complete care of children with brain tumors.
  • In addition, our brain tumor program is supported by specialized staff in nursing, rehabilitation services, neuropathology, pharmacy, neuroradiology, neuropsychology, ophthalmology, clinical nutrition, child life and social work, along with many others. All staff members have been specially trained to care for children with brain tumors.
  • St. Jude is committed to not only finding cures but also helping children with their quality of life. Children are not just small people. Their bodies and systems are unique, along with the disorders that affect them. For the best care, they need a specialized team. This is what you get from the St. Jude Brain Tumor Program.
  • St. Jude practices patient family-centered care. Patient family-centered care focuses on the family as a child’s primary source of strength, support and well being.
  • St. Jude is the only institution in the country to offer in-house clinical genomic testing, including whole genome sequencing of tumors, as a part of routine clinical care for patients. The results are used by our doctors to identify personalized treatment plans for each of our patients.

Contact the Brain Tumor Team any time, 24 hours a day, 7 days a week.

Call our Brain Tumor Coordinator, Tabatha E. Doyle, RN:
(901) 595-2544

Or email us at braintumors@stjude.org

Associated Clinical Trials

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

Phase II Study of Hippocampal-Avoidance Using Proton Therapy in Low-Grade Glioma

Diseases Treated:

 Low-grade glioma

Eligibility:

  • At least 6 years old and younger than 22
  • Diagnosis of low-grade glioma
View Trial

Loc3CAR: CAR T-Cell Therapy for Pediatric CNS Tumors

Locoregional Delivery of B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cells for Pediatric Patients with Primary CNS Tumors

Diseases Treated:

Brain tumors
DIPG

Eligibility:

  • Up to 21 years old
  • Either B7-H3–positive relapsed or refractory non-brainstem primary CNS tumor or brainstem high-grade neoplasm
View Trial

MEKPEM: A Phase I/II trial of MK-3475 (pembrolizumab) in children’s solid tumors and lymphomas

A Phase I/II Study of Pembrolizumab (MK-3475) in Children with Advanced Melanoma or a PD-L1 Positive Advanced Relapsed or Refractory Solid Tumor or Lymphoma (Merck study KEYNOTE-051, IND# 110,080, dated 10-15/2014). EudraCT NUMBER: 2014-002950-38

Diseases Treated:

Advanced melanoma or PD-L1 positive advanced relapsed or refractory solid tumor or lymphoma or high-grade glioma

Eligibility:

  • Between 6 months and 18 years old with diagnosis of MSI-H solid tumor OR
  • Between 12 years and 18 years old with diagnosis of melanoma OR
  • Between 3 and 18 years old with relapsed or refractory classical Hodgkin lymphoma OR
  • Between 12 and 18 years old with a diagnosis of Stage IIB, IIC, III, or IV melanoma, who had prior surgery to remove the tumor, but no other treatment (including radiation), no metastatic disease, and who have completely recovered. 
  • Negative pregnancy test 72 hours prior to medication administration in participants of child-bearing potential
  • Appropriate liver and kidney functions
View Trial

PBTC45: MK-3475 in Treating Children with Recurrent, Progressive or Refractory HGGs, DIPGs and Hypermutated Brain Tumors

A Safety and Preliminary Efficacy Trial of MK-3475 (Pembrolizumab; anti PD-1) in Children with Recurrent, Progressive or Refractory High-Grade Gliomas (HGG), DIPGs and Hypermutated Brain Tumors

Diseases Treated:

Recurrent, progressive, or refractory high-grade gliomas

Eligibility:

  • Participant has recurrent, progressive or refractory non-brainstem high-grade glioma or diffuse intrinsic pontine glioma (DIPG)
  • Participant is 1 to 18 years of age
  • Participant has bi-measurable disease on MRI 
  • Participant has received prior radiation therapy and/or chemotherapy and has not received myelosuppressive anticancer chemotherapy for at least three (3) weeks

 

View Trial

PBTC48: Study of the Optune Device in Children with Certain High-Grade Glioma and Ependymoma Brain Tumors

Feasibility Trial of Optune for Children with Recurrent or Progressive Supratentorial High-Grade Glioma and Ependymoma

Diseases Treated:

High-grade glioma (HGG), ependymoma

Eligibility:

  • Between 5 and 21 years old
  • Diagnosis of supratentorial high-grade glioma (HGG) or supratentorial ependymoma that is recurrent, progressive or refractory
  • Head circumference of at least 44 cm
  • Failed standard therapy with no other available treatment options
  • Recovered from prior chemotherapy, immunotherapy or radiotherapy
  • Willing and able to use the device at least 18 hours a day for at least 23 days and keep head shaved throughout treatment
View Trial

PBTC49: Study of Savolitinib in Recurrent, Progressive or Refractory Medulloblastoma, HGG or DIPG

Phase I study of Savolitinib in Recurrent, Progressive or Refractory Medulloblastoma, High-Grade Glioma, or Diffuse Intrinsic Pontine Glioma

Diseases Treated:

Medulloblastoma

High-grade glioma (HGG)

Diffuse intrinsic pontine glioma (DIPG)

Eligibility:

  • Recurrent, refractory or progressive medulloblastoma, high-grade glioma (HGG) or diffuse intrinsic pontine glioma (DIPG)
  • Evidence of genetic activation of the MET pathway (expansion co-hort)
  • At least 5 years old and 21 years old or younger
View Trial

PEPN2111: Study of CBL0137 in Patients with Relapsed or Refractory Solid Tumors and Lymphoma

A Phase 1/2 Trial of CBL0137 in Patients with Relapsed or Refractory Solid Tumors including CNS Tumors and Lymphoma

Diseases Treated:

Solid Tumor

Lymphoma

Brain Tumor

Eligibility:

  • 12 months to 21 years old with diagnosis of:
    • Relapsed or refractory solid tumor or lymphoma (including CNS tumors) OR
    • Progressive or recurrent DIPG or other H3 K27M-mutant diffuse midline gliomas previously treated with radiation therapy
  • 12 months to 30 years old with diagnosis of relapsed or refractory osteosarcoma
View Trial

SJ901: Study of Mirdametinib as a Treatment for Children, Adolescents and Young Adults with Low-Grade Glioma

Phase 1/2 Evaluation of Mirdametinib, a Brain-Penetrant MEK 1/2 Inhibitor, for Children, Adolescents and Young Adults with Low-Grade Glioma

Diseases Treated:

Low-Grade Glioma

Eligibility:

At least 2 years old and younger than 25 years old

Diagnosis of progressive or relapsed low-grade glioma 

View Trial

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