Gliomatosis cerebri is a rare brain cancer that strikes primarily children and adolescents, but has been diagnosed in adults as well. The cancer is a highly aggressive glioma that is very resistant to treatment. Gliomatosis cerebri does not consist of lumps like other tumors; it is made up of cancerous threads that spread quickly and infiltrate deep into the surrounding brain tissue and into multiple parts of the brain simultaneously. That makes this cancer very difficult to treat with either surgery or radiation. Gliomatosis cerebri is extremely rare, with fewer than 100 cases diagnosed in the United States each year.
Gliomatosis cerebri is an astrocytoma, which is the most common kind of glioma. An astrocytoma develops from a star-shaped type of glial cell called an astrocyte. Since glial cells are found throughout the brain and spine, these tumors can occur in a wide variety of locations throughout the central nervous system. Gliomatosis cerebri occurs most often in the frontal lobes of the cerebral hemispheres and by definition occurs in several lobes simultaneously.
What Causes Gliomatosis Cerebri?
Scientists do not yet understand what causes gliomatosis cerebri, and the cancer is so rare that it hasn’t received nearly enough research funding to find a cause, a cure, or even better treatment options. Survival rates have not improved much over the last few decades due to this lack of new research. Because of this, gliomatosis cerebri was chosen as one of the rare and inoperable pediatric brain tumors now being studied in the Weill Cornell Pediatric Brain and Spine Center’s Children’s Brain Tumor Project.
Diagnosing and Treating Gliomatosis Cerebri
Gliomatosis cerebri can be difficult to diagnosis because it is so diffuse. 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. A neurosurgeon may perform a surgical biopsy to extract a sample of the tumor for a positive diagnosis.
The prognosis for gliomatosis cerebri remains poor, but there are treatments that can prolong life and preserve function.
- Chemotherapy may be used to help shrink a tumor, or as follow-up after surgery to kill off any cancer cells left behind.
- Radiation therapy may help control the growth of the tumor and may be used instead of or in addition to surgery.
- 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 Gliomatosis Cerebri Registry
If you or someone you love has been diagnosed with gliomatosis cerebri, please visit our international registry at gcregistry.com for more information and helpful resources.
Research Into Gliomatosis Cerebri
Dr. Jeffrey Greenfield of Weill Cornell Pediatric Brain and Spine Center is conducting innovative research on gliomatosis cerebri. Genomic sequencing has allowed researchers to gather unprecedented amounts of data about gliomatosis cerebri; in Dr. Greenfield’s lab, researchers are studying that data in search of new treatment options.
Elizabeth’s brain was the very first gliomatosis cerebri sample to be sequenced. Since then, support from Elizabeth’s Hope has allowed Dr. Greenfield’s researchers to perform genomic sequencing and analysis on several other samples and the team has been successful in creating xenograft mouse models on which new agents and combinations of agents are being tested. Find out more about this exciting work by visiting childrensbraintumorproject.org or signing up for their newsletter below.