Gliomatosis cerebri is a very rare, fast-growing cancer of the brain. A main feature of gliomatosis cerebri is that it involves at least 3 lobes of the brain.
Gliomatosis cerebri is a rare, fast-growing cancer of the brain. It is a type of high-grade glioma. Unlike some cancers that form distinct, well-defined tumors, gliomatosis cerebri spreads throughout the brain. Gliomatosis cerebri involves at least 3 lobes of the brain.
Gliomatosis cerebri may occur at any age. In childhood, it most often occurs in older children and teens.
Gliomatosis cerebri does not respond well to treatment. Prognosis is usually poor even with intense therapy. Families may consider clinical trials, which test new treatments to see if outcomes can be improved.
Treatment for gliomatosis cerebri usually includes radiation therapy and chemotherapy. It is not possible to remove the cancer through surgery.
Early palliative care is important to help families manage symptoms and promote quality of life.
Symptoms of gliomatosis cerebri
Signs and symptoms of gliomatosis cerebri depend on the child’s age and where the tumor is located. Symptoms can be hard to predict. This is because of the way the cancer grows and spreads into brain tissue.
Gliomatosis cerebri symptoms may include:
Seizures
Headache
Nausea and vomiting
Changes in personality or behavior
Changes in memory or thinking
Vision problems
Loss of balance or problems walking
Fatigue or changes in activity levels
Weakness, numbness, tingling, or changes in feeling on one side of the body
Risk factors for gliomatosis cerebri
Gliomatosis cerebri may occur at any age. In childhood, it most often occurs in people who are 10–20 years old. The median age at diagnosis is 12 years.
There is no known cause of gliomatosis cerebri. But researchers are learning more about gene changes linked to these tumors.
Diagnosis of gliomatosis cerebri
Tests to diagnose gliomatosis cerebri may include:
A physical exam and health history to learn about symptoms, general health, past illness, and risk factors.
A neurological exam to assess brain function. This includes memory, vision, hearing, muscle strength, balance, coordination, and reflexes.
Imaging tests help identify the tumor, its size, and location. Magnetic resonance imaging (MRI) is the main imaging test. Gliomatosis cerebri can be hard to see on scans because the tumor does not have a clearly defined border. MRI is the preferred imaging method because it can better detect changes in the brain.
A lumbar puncture looks for cancer cells in the spinal fluid.
A biopsy helps diagnose gliomatosis cerebri. In a biopsy, a surgeon takes a small sample of the tumor. A pathologist looks at the tissue sample under a microscope to identify the type of tumor cells.
Genetic testing of the tumor to find specific changes (mutations) to identify the type of tumor and show possible targets for treatment.
Imaging features of gliomatosis cerebri
This MRI scan shows gliomatosis cerebri that affects both sides of the brain.
Gliomatosis cerebri is a subtype of glioma that can be used to describe how the tumor grows. It is not a formal diagnosis in the new WHO brain tumor classification.
MRI scans can identify gliomatosis cerebri pattern of growth. The spread of disease often includes the corpus callosum, brain tissue that connects the right and left brain. This means the tumor may cross over into both sides of the brain.
Gliomatosis cerebri may look like other non-cancer diseases that affect the brain. It is important to seek medical care from a major brain tumor center for correct diagnosis and treatment.
Brain diseases that can look like gliomatosis cerebri include:
Acute disseminated encephalomyelitis (ADEM)
Multiple sclerosis (MS)
Viral encephalitis
Leukodystrophies
Inherited myelin disorders
Imaging tests, biopsy, and other test results can provide information for diagnosis.
Grading and staging of gliomatosis cerebri
Gliomatosis cerebri does not follow a standard staging system. But the way the cells look under the microscope (histology) can help reveal the grade of disease.
The more aggressive the tumor cells look, the higher the grade. Higher grade tumors grow faster and are more likely to spread.
Health care providers use MRI or CT scans to classify the type of gliomatosis cerebri. There are 2 types. Both involve spread of disease, but there are differences based on imaging:
Type 1: There is no distinct tumor mass.
Type 2: A distinct, well-defined tumor mass is seen.
Scientists are studying gene changes in cancer cells to understand how tumors grow and to help plan treatments. Certain genetic features have been found in some pediatric gliomas, including gliomatosis cerebri. A gene mutation known as an IDH-mutation may be seen in different types of gliomas.
Genes being studied include IDH1R132H, H3F3AG34, CDKN2A, and PDGFRA.
Treatment of gliomatosis cerebri
There is no standard treatment plan for gliomatosis cerebri. Treatments may include radiation therapy, chemotherapy, and targeted therapy. Much of patient care focuses on controlling symptoms and supporting quality of life. Factors that influence treatment include:
How far the disease has spread
Location of the tumor
Genetic mutations of the tumor (IDH1 or IDH2 mutations)
Child’s age
Goals of care
Patients may be offered treatment with a clinical trial.
About half of patients will show a temporary response to therapy. This may include decrease in tumor size or improved symptoms and quality of life.
Radiation therapy for gliomatosis cerebri
Radiation therapy to the brain and spine is a main treatment for gliomatosis cerebri. Patients may have some temporary improvements in symptoms. Treatment may extend life. But radiation therapy is not a cure. A large area of radiation can have a higher risk of side effects.
Chemotherapy for gliomatosis cerebri
Chemotherapy is often used along with radiation therapy or separately.
Chemotherapy is based on the type and grade of the tumor. Chemotherapy used for gliomatosis cerebri includes:
Other types of chemotherapy may also be used, especially within a clinical trial.
Targeted therapies for gliomatosis cerebri
Some tumors have specific genetic changes or mutations in the tumor cells. These gene changes can sometimes be treated with special medicines called targeted therapies. Targeted therapies work by acting on, or targeting, specific features of tumor cells. Targeted therapies for IDH1 or IDH2 mutation may be an option for some patients.
Surgery for gliomatosis cerebri
Surgery is not a main treatment for gliomatosis cerebri. It is not possible to remove the tumor due to widespread disease that affects multiple brain areas. Surgery may be used for diagnosis or to help with symptoms of the tumor. In some cases, surgery may help relieve pressure on the brain.
Prognosis for gliomatosis cerebri
Gliomatosis cerebri is an aggressive, fast-growing brain cancer. The tumor resists current therapies. This means the prognosis is usually poor. There is no long-term cure.
Among children and teens, the average time of survival after diagnosis of gliomatosis cerebri is about 1.5 years in the United States.
Factors that influence prognosis for gliomatosis cerebri include:
Type and grade of the tumor: High-grade tumors have a worse prognosis.
How much the cancer has spread at diagnosis: Greater spread of disease is linked to worse outcome.
Molecular or genetic features of the tumor: Scientists are studying whether certain changes in genes and cell features of the tumor can help your care team plan specific treatments. For example, patients who have tumors with IDH1/2 mutations have a better prognosis.
Age of the patient: Younger patients with gliomatosis cerebri tend to have a better outcome.
Poor response to initial therapy: Tumors that do not respond to early treatment are harder to treat.
Symptoms that get worse during therapy: Patients with these changes have a worse prognosis.
Thinking, learning, or behavioral changes at diagnosis are linked to a worse outcome.
Support for patients with gliomatosis cerebri
Patients may show short-term improvement with treatment. But there is no long-term cure for gliomatosis cerebri. Families should talk to their care teams about problems they might expect as the disease progresses.
Many patients with gliomatosis cerebri have seizures during their illness. Anti-seizure medicines can help control this symptom. Your neurologist can also help manage seizures.
Headaches are another common symptom of gliomatosis cerebri. The tumor may cause hydrocephalus. This causes increased pressure inside the brain. This may be managed with steroid medications or surgery to place a shunt.
A shunt is a small tube that drains cerebrospinal fluid from the brain. The shunt may be short-term or permanent. This depends on what the patient needs for symptom control.
The long-term prognosis of gliomatosis cerebri is poor. Talk to your care team as soon as possible about goals of therapy and quality of life. These conversations should begin early and continue throughout the care process.
A team of health care providers can help meet specific patient needs as the disease progresses:
High-grade gliomas are cancerous brain tumors that can grow and spread quickly. Learn about symptoms, diagnosis, and treatment of pediatric high-grade gliomas.
