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Gliomatosis Cerebri Overview

Gliomatosis cerebri is a rare diagnosis of brain cancer. As it most commonly presents itself as a diffusely infiltrating tumor involving more than one lobe of the brain, and with research reported out at the Gliomatosis Cerebri Conference at NIH in June 2017, it is now thought to be not a distinct type of tumor, but more a process that a tumor undertakes which makes it spread in a distinct way. It is typically a high grade glioma, which means it involves the “glial” cells or connective tissue of the brain.  

Sometimes Gliomatosis is considered a “mixed” glioma when the tumor contains both astrocytes ( a kind of star-like shaped glial cell) and oligodendrocytes ( another kind of glial cell), although this differentiation is no longer routinely classified.

It is generally considered a highly aggressive malignant tumor, which means it contains cancer cells, and is therefore a brain cancer. ( Benign tumors do not contain cancer cells ). Primary Gliomatosis Cerebri is more common in children, but does occur in adults also.

It differs from most brain cancers in the way it grows. There generally is no single tumor mass, but infiltrates through different parts of the brain as it grows. It has been described to us being similar to sand spilling, or milk spilling, where it spreads in and around other brain cells, instead of a single mass which grows larger in size. 

What Causes Gliomatosis Cerebri and How Common is it?

There are no known factors or conditions that make a person more or less likely to develop gliomatosis cerebri. It is not known to be an inherited condition and there are no known environmental factors.

Gliomatosis Cerebri is a very rare brain tumor. They represent less than 3 percent of pediatric brain tumors.

There are most likely less than 100 known cases of primary Gliomatosis Cerebri diagnosed each year.

How is Gliomatosis Cerebri Diagnosed?

Physical Exam
- Following an assessment of symptoms, and a physical exam,

which may include a neurological exam, a Physician may order one or more of the following diagnostic tests.

CT or CAT Scan  (computerized tomography scan) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general x-rays.

MRI (magnetic resonance imaging) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI provides greater anatomical detail than CT scan and does a better job of distinguishing between tumors, tumor-related swelling and normal tissue. Younger children may be given a sedative to help them remain still during the test. The MRI test is loud and sounds like banging during the test, which should be explained to the child. Parents are generally allowed to remain in the room.

MRS (magnetic resonance spectroscopy) - a test done along with an MRI. It can detect the presence of organic compounds within sample tissue that can identify the tissue as normal or tumor, and may also be able to tell if the tumor is a glial tumor or if it is of neuronal origin (originating in a neuron, instead of in a brain cell).

 Biopsy or Surgery

A biopsy may be performed to assist in diagnosis of the brain tumor, and to attempt to remove as much of the tumor as possible. As typically the tumor is widespread and diffuse, it is difficult to remove the tumor during surgery. 

Personalized Medicine

The concept of personalized medicine is to look at each brain tumor individually. Instead of grouping all of one type brain tumor together and treating them all the same, it looks at each tumor individually, identifying the specific mutations of that tumor, and determines a treatment based on the specific mutations, not by the type of tumor.

This concept is explained well in the following video by Dr Mark Kieran

Video – Dr Mark Kieran “Multi-disciplinary Approaches to Treating Pediatric Brain Tumors”

The following video link is to an hour long video which was recorded through the American Brain Tumor Association, and is available on their website and also on You Tube. It is not specific to Gliomatosis Cerebri, but It goes through a very clear description of the concept of personalized medicine and focus on mutations in brain tumors, and recent advances in treatment of brain cancers. When you have the time, I highly recommend it

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GC Research Articles

Gliomatosis cerebri: A consensus summary report from the First International Gliomatosis cerebri Group Meeting, March 26–27, 2015, Paris, France 

Gliomatosis Cerebri: Current Understanding and Controversies, by Surabhi Ranjan, Katherine E. Warren, published in Frontiers in Oncology, August 2017