Low-grade gliomas are brain tumors that originate from glial
cells, which support and nourish neurons in the brain. Glial tumors, or
gliomas, are divided into four grades, depending on their cells' appearance
under a microscope. Grade 1 and 2 gliomas are considered low-grade and account
for about two-thirds of all pediatric tumors.
In addition to their grade, low-grade gliomas are also
classified based on their location and by the kind of glial cell – astrocytes,
oligodendrocytes or ependymocytes – from which they arise.
Most low-grade gliomas are
both highly treatable and highly curable. The most common kind of low-grade
glioma, called a pilocytic astrocytoma, has a cure rate over 90 percent.
Our glioma specialists – a team of neuro-oncologists,
surgeons, pathologists and radiation oncologists – focus solely on the care of
children diagnosed with gliomas. The Glioma 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.
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:
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).