Gliomatosis cerebri can be difficult to diagnose because it is diffuse, or threadlike, and does not have clear borders. A patient with any neurological symptoms will first be given a physical exam that includes neurologic function tests (reflexes, muscle strength, eye and mouth movement, coordination and alertness). If a tumor is suspected, the patient will have imaging tests so that doctors can look into the brain for any abnormality. These tests may include:
Magnetic resonance imaging (MRI) and computerized tomography (CT) scans produce detailed images of the brain and spine and allow doctors to detect the presence of a tumor. Since gliomatosis has such poorly defined borders, it can be difficult to diagnose even with imaging tests.
A surgical biopsy may be performed to help confirm the diagnosis. In this procedure, a neurosurgeon extracts a small sample of abnormal cells to test in a pathology laboratory. At advanced medical centers, a neurosurgeon may remove enough tissue to perform “molecular characterization” of the tumor. This is like getting a fingerprint of the tumor, which allows the medical team to match a patient’s tumor with chemotherapy or targeted molecules that may better attack the tumor.
Although there is no cure for gliomatosis cerebri, and the prognosis is poor, there are treatments available at major medical centers, where a highly specialized team can provide innovative options.
Personalized medicine has been considered a hopeful option that requires a team with advanced training and skills who can develop an individualized treatment plan. An advanced health institute like the Weill Cornell Medicine Brain and Spine Center can take advantage of highly advanced capabilities like genetic sequencing and drug screening, to help determine the precise treatment plan for a particular tumor — an approach called personalized medicine.
Surgery may be considered for some patients to remove some of the tumor or to relieve pressure (see Surgery for Gliomatosis Cerebri). Other options include:
Chemotherapy may be used to help shrink a tumor, or as follow-up after surgery to kill off any cancer cells left behind. It is given systemically (meaning to the whole body, not just to the site of the tumor) and may be a pill, an injection, or an IV drip.
Radiation therapy may help control the growth of the tumor and may be used instead of or in addition to surgery. Precisely targeted radiation can be an alternative to surgery or can kill cancer cells left behind after surgery.
Surgery may be performed to extract a sample of the tumor for a biopsy, to remove as much of the tumor as possible, or to place or reposition a shunt. (See Surgery for Gliomatosis Cerebri.)
Other treatments may include steroid treatment to reduce swelling, or anti-seizure medication. Researchers are now investigating other treatments, including immunotherapy and gene therapy.
The prognosis for gliomatosis cerebri remains poor, but treatment can prolong life and preserve function while laboratory researchers continue to search for better alternatives. (Find out more about the Children’s Brain Tumor Project.)
Reviewed by: Jeffrey Greenfield, Ph.D., M.D.
Last reviewed/last updated: April 2021