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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.
Early palliative care is important to help families manage symptoms and promote quality of life.
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:
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.
Health care providers test for brain tumors in several ways. These tests may include:
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:
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.
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:
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 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 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.
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.
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:
Thinking, learning, or behavioral changes at diagnosis are linked to a worse outcome.
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:
Reviewed: November 2023