Gliomatosis Cerebri

What is gliomatosis cerebri?

Gliomatosis cerebri is a rare, fast-growing cancer of the brain. It is a type of 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

Health care providers test for brain tumors in several ways. These tests may include:

• A physical exam and medical history help your care team learn about:
  • Your child’s symptoms
  • General health
  • Past illness
  • Risk factors
• A neurological exam measures types of brain function including:
  • Memory
  • Vision
  • Hearing
  • Muscle strength
  • Balance
  • Coordination
  • Reflexes
• Imaging tests help identify the tumor, see how big the tumor is and whether it has spread, and find out what brain areas may be affected. Most patients will have both computed tomography (CT) and magnetic resonance imaging (MRI). 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. The images made by MRI can give more details about the type of tumor and the possible spread of disease.
• 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.

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. 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
• The 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

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

Surgery

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.
• 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:

• Palliative care or quality of life services help patients and families manage pain and other symptoms, promote quality of life, and navigate care decisions, including treatment choices.
• Hospice care provides medical and practical support as patients near the end of life.
• Child life, social work, spiritual care, and psychology can help with emotional and practical needs through the cancer journey for the entire family.

Questions to ask your child’s care team

• What are our treatment options?
• What are the possible side effects of each treatment?
• What can we expect as my child’s illness progresses?
• What can be done to manage my child’s symptoms?
• Will my child need to be in the hospital for treatment?
• What options should we consider to promote quality of life for my child?