Low-grade Glioma in Children

Low-grade gliomas are slow-growing tumors. As they grow, they press on surrounding healthy parts of the brain, affecting their function. As such, the symptoms of a pediatric low-grade glioma depend on the tumor's size and where in the brain it is located.

Some of the most common symptoms of a pediatric low-grade glioma include:

• headache, particularly in the morning or made better by vomiting
• severe or frequent vomiting without other signs of gastrointestinal illness
• vision problems, such as double vision, blurry vision or loss of vision
• difficulty walking or balancing
• seizures
• weight gain or loss
• premature puberty
• clumsiness
• confusion
• sleepiness
• changes in behavior

To diagnose a pediatric low-grade glioma, your doctor will take your child's medical history and carry out both physical and neurological exams. Your doctor may also order a variety of tests, including:

• a magnetic resonance imaging (MRI) scan to evaluate the tumor and determine its extent
• biopsy or tissue sample from the tumor to provide definitive information about the type of tumor and its grade, and allow for molecular profiling of tumor cells
• electroencephalogram (EEG), which measures the brain's electrical activity
• lumbar puncture, in which a doctor takes a small sample of cerebrospinal fluid from near the base of the spine to see whether the tumor has spread

Specific low-grade glioma diagnoses could include:

• astrocytoma
• pilocytic astrocytoma
• fibrillary astrocytoma
• angiocentric glioma
• dysembryoplastic neuroepithelial tumor (DNET or DNT)
• ganglioglioma and glial neuronal tumors
• oligodendroglioma
• optic nerve (pathway) glioma
• pleomorphic xanthoastrocytoma (PXA)
• brainstem tumors, including diffuse pontine glioma (DIPG) and tectal glioma
• thalamic and hypothalamic astrocytoma

After all tests are completed, doctors will be able to outline the best treatment options.

Our treatment approach for pediatric gliomas is personalized for each patient depending on several factors, including the tumor's type, stage and location. Some therapies will treat the tumor while others are intended to address complications of the disease or side effects of the treatment.

In addition, our clinicians may offer access to targeted treatments based on the molecular profile of your child's tumor.

Some of the options your doctor may discuss include:

• surgery to remove as much of the tumor as possible
• chemotherapy, either before surgery to shrink the tumor or after surgery to eliminate any remaining tumor cells
• radiation therapy; while this was once the standard of care, this option is now generally avoided, if possible, for children with pediatric low-grade gliomas to avoid possible long-term side effects

We also offer innovative brain tumor clinical trials for children with low-grade gliomas. Some of these were launched by our own physicians, while others are available through our participation in collaborative groups such as the Children's Oncology Group (COG) and the Pacific Pediatric Neuro-Oncology Consortium (PNOC).

Should you have questions or need advice on whether a particular trial would be appropriate for your child, email our clinical trials team at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options.

Your child’s prognosis (chance of recovery) and treatment options depend on a number of different factors, including:

• type of tumor
• tumor grade
• the extent of the disease
• the size and location of the tumor
• the presence or absence of metastasis
• the tumor's response to therapy
• the age and overall health of your child
• your child's tolerance of specific medications, procedures or therapies
• new developments in treatment

In general, low-grade gliomas tend to be readily treatable. Prompt medical attention and appropriate therapy are important for the best prognosis.

Many brain tumor survivors face physical, psychological, social and intellectual challenges related to their treatment and will require ongoing assessment and specialized care.

To address the needs of this growing community of brain tumor survivors, Dana-Farber/Boston Children's established the Stop & Shop Family Pediatric Neuro-Oncology Outcomes Clinic. This multi-disciplinary program addresses long-term health and social issues for families and survivors of childhood brain tumors.

Today, the Outcomes Clinic follows more than 1,000 pediatric brain tumor survivors of all ages, providing such services as:

• MRI scans to monitor for tumor recurrences
• intellectual function evaluation
• endocrine evaluation and treatment
• neurologic assessment
• psychosocial care
• hearing, vision monitoring
• ovarian dysfunction evaluation and treatment
• motor function evaluation and physical therapy
• complementary medicine

As a result of treatment, children may experience changes in intellectual and motor function. Several programs address these needs, among them the School Liaison and Back-to-School programs, which provide individualized services to ease children's return to school and maximize their ability to learn. In addition to providing thorough and compassionate care, Outcomes Clinic specialists conduct innovative survivorship research and provide continuing education for staff, patients and families.

Research is a top priority at Dana-Farber/Boston Children's, and our physicians work continuously to translate laboratory findings into clinical therapies and find ways to improve survival while reducing the toxicity and long-term impact of treatment. 

For instance, a 2014 study led by Peter Manley, MD, documented the excellent long-term survival among patients with low-grade gliomas, and the negative impact of radiation therapy—long a mainstay of pediatric brain tumor treatment—on that survival.

The Glioma Program's research enterprise mirrors its clinical efforts in its multidisciplinary nature. Basic, translational and clinical scientists in the program work together and with colleagues at institutions like the Broad Institute to uncover new knowledge about the biology of gliomas and translate that understanding into new therapies or ways of overcoming resistance to existing ones.

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 Mark Kieran, MD, PhD, clinical director of the Brain Tumor Center at Dana-Farber/Boston Children's, and 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 growth of low-grade gliomas. Specifically, we have identified genes that are commonly mutated in low-grade glioma. These findings are guiding clinical trials examining the activity of new drugs specifically for children with low-grade glioma.

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).