Gliomatosis Cerebri

Also called: Cerebri gliomatosis multiforme

What is gliomatosis cerebri?

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

Gliomatosis cerebri 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 extends throughout brain tissue, it is not possible to remove the cancer through surgery.

Gliomatosis cerebri as a type of glioma

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. In other words, gliomatosis cerebri is not a formal diagnosis but is used to describe how the tumor grows. This rare pattern of growth may be seen in gliomas such as astrocytoma.

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.

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 because of the way the cancer grows and spreads out 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

Diagnosis of gliomatosis cerebri

Doctors test for brain tumors in several ways. These tests may include:

A physical exam and medical history help 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 measures 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, if it has spread, and find out what brain areas may be affected. Most patients will be evaluated using both computed tomography (CT) and magnetic resonance imaging (MRI). Gliomatosis cerebri can 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 of the brain. The images made by MRI can give more information about the type of tumor and the potential spread of disease.
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.

Imaging features of gliomatosis cerebri

Doctors use MRI and CT scans to identify gliomatosis cerebri pattern of growth. The spread of disease often affects both hemispheres of the brain and includes the corpus callosum, brain tissue which bridges the right and left hemispheres of the brain. In other words, the tumor may cross over into both sides of the brain. Unlike in other brain tumors, the underlying structure of brain tissue often remains stable. This is especially true in Type I tumors where there is no distinct tumor mass. Type II tumors have a more obvious and defined mass as well as diffuse spread.

Gliomatosis cerebri may look like other non-cancer diseases that affect the brain. These include acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), viral encephalitis, leukodystrophies, and heritable myelin disorders. Imaging features and biopsy provide information for diagnosis.

MRI scan showing gliomatosis cerebri pattern of growth crossing hemispheres of the brain.

Gliomatosis cerebri is a diffuse tumor and spreads out into brain tissue.

Grading and staging of gliomatosis cerebri

Gliomatosis cerebri is diffuse or spread out at diagnosis. It doesn’t follow a standard staging system. However, the way 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 tumors are most often classified as WHO Grade III high-grade glioma, but they can also be Grade II or IV.

Doctors also use MRI or CT scans to classify the type of gliomatosis cerebri. There are 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, well-defined 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. 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.

Gliomatosis cerebri prognosis

Gliomatosis cerebri is an aggressive, fast-growing cancer. The tumor is resistant to current therapies, and the prognosis is poor. There is no long-term cure.

Factors that influence prognosis for gliomatosis cerebri include:

Type and grade of the tumor. High-grade tumors have a worse prognosis. Patients with oligodendroglioma tumors may have a longer survival compared to astrocytoma tumors.
How much the cancer has spread at diagnosis. Greater spread of disease is associated with 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 doctors 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.
Changes in memory, thinking, or behavior at diagnosis. Cognitive problems or behavioral changes at diagnosis and progression of problems during therapy are associated with worse outcome.

Among children and teens, the average time of survival after diagnosis of gliomatosis cerebri is about one and a half years.

Treatment of gliomatosis cerebri

Gliomatosis cerebri is a rare, aggressive brain tumor. Currently, there is no standard treatment plan. Much of patient care is focused on controlling symptoms and supporting quality of life for as long as possible. 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 with a clinical trial.

About half of patients will show some temporary response to therapy. Treatment response may include decrease in tumor size or clinical improvements in symptoms and improved 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, and treatment may extend life. However, radiation therapy is not curative. The diffuse nature of the tumor also makes radiation therapy more complicated. A large area of radiation can have a higher risk of side effects.

Radiation therapy may help slow or shrink gliomatosis cerebri and relieve symptoms.
Chemotherapy

Chemotherapy is often used along with radiation therapy or separately. Chemotherapy is planned based on the type and grade of the tumor. Chemotherapy used for gliomatosis cerebri includes procarbazine, lomustine (CCNU), and vincristine (PCV regimen), and temozolomide. Other types of chemotherapy may also be used, especially within a clinical trial.
Surgery

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. In some cases, surgery may be used to remove parts of the tumor to relieve pressure on the brain.

Supportive care for gliomatosis cerebri

Patients may show temporary improvement with therapy. However, there is no long-term cure. 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. Anti-seizure medications can help to control this symptom. Neurology specialists also assist with the management of seizures.

Headaches are another common symptom of gliomatosis cerebri. The tumor may cause hydrocephalus, 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 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.

The long-term prognosis of gliomatosis cerebri is poor. Conversations around goals of therapy and quality of life should begin early in 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 and end of life care. 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.

More: Life After Brain Tumors

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Reviewed: February 2020