Glioblastoma

Other Names: Glioblastoma multiforme

Glioblastoma is a malignant (cancerous) brain tumor that develops from a specific type of brain cell called an astrocyte. These cells help support and nourish neurons (nerve cells of the brain) and form scar tissue that helps repair brain damage in response to injury. Glioblastomas are often very aggressive and grow into surrounding brain tissue.

Glioblastoma (histology slide)

Axial post-contrast T1 (top) and T2 (bottom) weighted MRI showing an IDH1 mutant frontal lobe glioblastoma with sparse enhancement despite large size.

Cause

In most cases, the exact underlying cause of glioblastoma is unknown. In rare cases, they can occur in people with certain genetic syndromes such as neurofibromatosis type 1, Turcot syndrome and Li Fraumeni syndrome. In these cases, affected people usually have other characteristic features of the condition that are all caused by changes (mutations) in a specific gene

Inheritance

Most glioblastomas are not inherited. They usually occur sporadically in people with no family history of tumors.

Risk factors

Glioblastoma can rarely occur in people with certain genetic syndromes such as neurofibromatosis type 1, Turcot syndrome and Li Fraumeni syndrome. All of these conditions are inherited in an autosomal dominant manner.

Symptoms

Signs and symptoms of glioblastoma vary depending on the size and location of the tumor but may include:

• Headache
• Nausea and vomiting
• Drowsiness
• Changes in personality
• Weakness on one side of the body
• Memory loss
• Speech difficulty
• Changes in vision
• Seizures

Diagnosis

GBM in the frontal right lobe as seen on CT scan

Sagittal MRI with contrast of a glioblastoma WHO grade IV in a 15-year-old boy

Genetic testing is not available for many people with glioblastoma since most of these tumors occur sporadically (by chance) and are not caused by a genetic mutation. However, genetic testing is an option for people with an inherited condition that predisposes to glioblastoma such as neurofibromatosis type 1, Turcot syndrome and Li Fraumeni syndrome.

Glioblastoma is typically diagnosed based on a physical exam that identifies characteristic symptoms and various imaging studies such as computed tomography (CT) and/or magnetic resonance imaging (MRI). A CT scan is an imaging method that uses x-rays to create pictures of cross-sections of the body, while an MRI scan uses powerful magnets and radio waves to create pictures of the brain and surrounding nerve tissues. These imaging studies will also provide information regarding the size of the tumor and which parts of the brain are affected. Surgical removal of the tumor or a small biopsy may confirm the diagnosis

Treatment

Unfortunately, there is no cure for glioblastoma. Treatment is palliative and may include surgery, radiation therapy and/or chemotherapy. The best treatment options for each person depend on many factors including the size and location of the tumor; the extent to which the tumor has grown into the surrounding normal brain tissues; and the affected person's age and overall health. Glioblastoma is often treated with surgery initially to remove as much of the tumor as possible. In most cases, it is not possible to remove the entire tumor so additional treatment with radiation therapy and/or chemotherapy is necessary.

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition.

• Bevacizumab (Brand name: Avastin) Treatment of adult patients with recurrent glioblastoma. Also treatment of glioblastoma with progressive disease following prior therapy.
• Polifeprosan 20 with carmustine (Brand name: Gliadel®)As an adjunct to surgery to prolong survival in patients with recurrent glioblastoma multiforme for whom surgical resection is indicated

Prognosis

There is currently no cure and despite treatment, many affected people live less than a year after the initial diagnosis. Younger age; higher Karnofsky performance (a standard measure of the ability of patients with cancer to perform daily tasks) score at diagnosis; and treatment with chemotherapy and radiation therapy are all associated with a better prognosis. The extent to which the tumor is able to be removed also appears to influence the outcome.

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NIH genetic and rare disease info

• NIH info on Glioblastoma

Glioblastoma is a rare disease.