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Gliomatosis Cerebri

Also called: Cerebri gliomatosis multiforme

What is Gliomatosis Cerebri?

Gliomatosis cerebri is a very rare, fast-growing cancer of the brain. Unlike some cancers that form distinct, well-defined tumors, gliomatosis cerebri is diffuse and spreads out into brain tissue. This cancer is a type of glioma, but it involves at least 3 lobes of the brain. 

Gliomatosis cerebri is an aggressive cancer and does not respond well to treatment. Even with intensive therapy, prognosis is poor. Treatment for gliomatosis cerebri usually includes radiation therapy and chemotherapy. Because this cancer is widespread and infiltrates brain tissue, it is not possible to remove the cancer through surgery.

Radiation therapy uses beams of radiation, X-rays or protons, to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

Radiation therapy uses beams of radiation, X-rays or protons, to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

In the past, gliomatosis cerebri was considered a separate type of central nervous system (CNS) tumor. However, the most recent World Health Organization (WHO) classification of tumors includes gliomatosis cerebri as a subtype of glioma with a distinct growth pattern or special pattern of spread.

Gliomatosis cerebri may occur at any age. In childhood, it most often occurs in older children and adolescents, 10-20 years of age. The median age at diagnosis is 12 years.

Signs and Symptoms of Gliomatosis Cerebri

Signs and symptoms of gliomatosis cerebri depend on the child’s age and tumor location. Symptoms can be hard to predict because of the way the cancer grows and spreads out into brain tissue.

Gliomatosis cerebri symptoms may include:

  • Seizures
  • Headache
  • Changes in personality or behavior
  • Changes in memory or cognitive abilities
  • Problems with vision
  • Loss of balance or problems walking
  • Fatigue, changes in activity levels
  • Weakness, numbness, tingling, or changes in feeling on one side of the body

Diagnosis of Gliomatosis Cerebri

Doctors test for brain tumors in several ways.  

  • A health history and physical exam helps doctors learn about symptoms, general health, past illness, and risk factors. 
    • There is currently no known cause of gliomatosis cerebri. However, researchers are learning more about gene changes associated with these tumors. 
  • A neurological exam assesses function of the brain, spinal cord, and nerves. These tests measure different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes. 
  • Imaging tests are used to help identify the tumor, see how big the tumor is and if has spread, and find out what brain areas may be affected. Most patients will be evaluated using both computed tomography (CT Scan) and magnetic resonance imaging (MRI). Gliomatosis cerebri can sometimes be difficult 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 white matter. The images made by MRI can give more information about the type of tumor and the potential spread of disease.

In gliomatosis cerebri, MRI helps doctors visualize spread of disease including bilateral infiltration and invasion of the corpus collosum (brain tissue which bridges the right and left hemispheres of the brain). Unlike in some other brain tumors, the underlying structure of brain tissue often remains stable. This is especially true in Type I tumors where a distinct tumor mass is not present. Type II tumors are characterized by a more obvious mass in addition to the diffuse component.

Gliomatosis cerebri may resemble other conditions including acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), viral encephalitis, leukodystrophies, and heritable myelin disorders. Imaging features provide more information important for diagnosis.

  • A lumbar puncture may be performed to look for cancer cells in the spinal fluid.
  • A biopsy is performed to help diagnose gliomatosis cerebri. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type of tumor cells.
Axial MRI with markings to indicate gliomatosis cerebri

Axial MRI with markings to indicate gliomatosis cerebri

Axial MRI with markings to indicate gliomatosis cerebri

Gliomatosis cerebri shown in axial MRI

Grading and Staging of Gliomatosis Cerebri

Gliomatosis cerebri is diffuse or spread out at the diagnosis. It doesn’t follow typical cancer staging. However, how the cells look under the microscope (histology) can give information about 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. Gliomatosis cerebri are most often classified as WHO Grade III glioma but can also be Grade II or IV.

Gliomatosis cerebri can be divided into two types based on imaging:

  • Type I – There is diffuse spread of disease and infiltration of brain tissue, but there is no distinct tumor mass.
  • Type II – A distinct tumor mass is seen, and there is diffuse spread of disease.

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. Genes being studied include IDH1R132H, H3F3AG34, CDKN2A, and PDGFRA.

Prognosis of Gliomatosis Cerebri

Gliomatosis cerebri is an aggressive, fast-growing cancer. The tumor is resistant to current therapies, and the prognosis is poor. Factors that influence prognosis include:

  • Molecular and genetic features of the tumor
  • Histological features including tumor grade and markers that predict response to treatment 
  • Age (Younger patients tend to have a better outcome.)
  • Poor response to initial therapy
  • Extent of disease at diagnosis  
  • Change in cognitive and behavioral function at diagnosis and progression of problems during therapy

Higher grade tumors and more advanced disease are associated with worse outcome. 

Average time of survival after diagnosis of gliomatosis cerebri is 17 months in pediatric patients.

Treatment of Gliomatosis Cerebri

Gliomatosis cerebri is a rare, aggressive cancer, and there is no standard treatment plan. Factors that influence treatment options include extent of disease, location of the tumor, the child’s age, and goals of care. Patients may be offered treatment within a clinical trial.

About half of patients will show some response to therapy. Therapeutic response may include decrease in tumor size and/or clinical improvements in symptoms and improved quality of life.

  1. Radiation therapy to affected areas in the brain and spine is a main treatment for gliomatosis cerebri. Patients may have some temporary improvements in symptoms, and treatment may extend life. However, radiation therapy is not curative.

  2. Chemotherapy is often used along with radiation therapy or separately. Chemotherapy is usually given systemically either by oral or IV. Chemotherapy is planned based on the grade of the tumor.

  3. Surgery is not a main treatment for gliomatosis cerebri. It is not possible to remove the tumor due to widespread disease and impact on multiple brain areas.

    Surgery may be performed as part of a biopsy for diagnosis or to help with symptoms of the tumor.

Supportive Care for Gliomatosis Cerebri

Although patients may show temporary improvement with therapy, gliomatosis cerebri is fast-growing and progressive. Families should talk to their care teams about what problems they might expect as the disease progresses. Many patients with gliomatosis cerebri experience seizures during the course of their illness. Appropriate use of anti-seizure medication can help to control this symptom. Neurology specialists also assist with the management of seizures. Headaches are another common symptom and may be due to increased intracranial pressure, or pressure inside the brain. This may be managed with steroid medications or surgical placement of a shunt. A shunt is a small tube that drains cerebrospinal fluid so that it can be removed from the brain. The shunt may be temporary or permanent, depending on what the patient needs for symptom control.

These illustrations show a girl with a shunt placed. The different versions show what the shunt looks like when placed towards the front of the body and towards the back of the body. The illustration is labeled to identify parts of the device: valve, catheter, tubing. A ventricle in the brain and the peritoneal space where the shunt drains are also labeled.

A shunt is a small tube that drains cerebrospinal fluid to prevent the build-up of fluid.

Given the poor prognosis of gliomatosis cerebri, conversations around goals of therapy and quality of life are also important. These discussions should begin early in the care process. Incorporating palliative care or quality of life services can help patients and families manage symptoms, promote quality of life, and navigate care decisions.

More: Life After Brain Tumors

Reviewed: June 2018