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With a serious diagnosis comes lots of questions; ones you want answers to fast. You also need information you can trust in a language that doesn't require an advanced medical degree to understand.
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What Is Gliomatosis Cerebri?
Gliomatosis cerebri is a rare type of brain cancer 1. Specifically, it is a tumor in the glial (connective) cells which affects the central nervous system (CNS).
Tumors in the central nervous system begin in the brain or spinal cord. Gliomatosis cerebri is not recognized as a formal diagnosis anymore. Instead, gliomatosis cerebri refers to a specific pattern of diffuse and extensive growth of glioma cells.
This type of tumor in the glioma cells invade multiple lobes of the brain. There are different types of grades and cells of origin that can grow in this specific pattern.
Although more research is being done, very little is known about the origin of this disease and what the best route of treatment is.
Gliomatosis cerebri is closely related to glioblastoma multiforme, a deadly tumor that primarily occurs in adults. Glioblastoma multiforme is the same tumor that U.S. Senator Edward Kennedy and star baseball player Gary Carter suffered from.
What Are the Grades of Gliomatosis Cerebri?
If you are unfamiliar with the grading scale of tumors 2 in general, this next portion may help you understand the severity of this type of cancer.
- Grade I: The tissue in a Grade I tumor is benign. This is the only grade of tumor that is not considered cancerous. Cells affected by this low-grade tumor look nearly like normal brain cells. These tumors also grow very slowly.
- Grade II: The tissue in a Grade II tumor is malignant (or cancerous). These low-grade tumorous cells look less like normal cells and grow a little bit faster than grade I tumors.
- Grade III: The tissue in a Grade III tumor is malignant. The tissue in this high-grade tumor has cells that look very different from normal cells. These different or abnormal-looking cells are actively growing.
- Grade IV: The tissue in a Grade IV tumor is malignant. The cells in the tissue of this high-grade IV tumor look the most abnormal and generally grow very rapidly.
Overall, the difference between low-grade tumors and high-grade gliomas is that the lower grade (grades I and II) look more normal and grow slower than high-grade tumors (Grades III and IV).
Gliomatosis cerebri always presents itself in one of the three malignant grades (grades II, III or IV).
Signs and Symptoms of Gliomatosis Cerebri
Signs and symptoms that present with gliomatosis cerebri tend to vary depending on the location and severity of the tumor growth. There are no “classical symptoms” due to the different locations.
However, people with gliomatosis cerebri may commonly present symptoms such as focal weakness, seizures, sensory loss, progressive headache, memory loss or dementia-like symptoms, visual deficits, increased intracranial pressure, developmental delays in children, tremors and nose bleeds.
Due to the severity and poor prognosis of this type of cancer, many who are diagnosed will not recover and will enter end-stage glioblastoma.
During this stage, the priority of physicians is to minimize pain and provide the highest quality of life possible.
Symptoms that patients have during this stage include drowsiness, incontinence, seizures, bodily pain, headaches, progressive cognitive deficits, difficulty swallowing and confusion. This, however, is monitored closely by the healthcare team and symptoms are minimized if possible 3.
Causes of Gliomatosis Cerebri
There is no known cause of gliomatosis cerebri. However, when the brain or spinal cord cells are injured through stress, trauma or disease, glial fibrillary acidic protein is released rapidly by astroglial cells.
These cells have the responsibility of supporting and nourishing the cells in both the brain and spinal cord.
Although the function of this glial fibrillary acidic protein is not fully understood, it is thought to play a role in controlling the function, movement and shape of astroglial cells 4.
Is Gliomatosis Cerebri Hereditary?
A question that is often a concern is, “can gliomatosis cerebri be passed through families?” There has not been enough research to suggest that gliomatosis cerebri is hereditary.
However, some people think that it is possible for this type of cancer to run in families as other cancers do.
How Common Is Gliomatosis Cerebri?
Gliomatosis Cerebri is a very rare form of brain cancer.
There are only an estimated 247 people living with this condition currently in the world. There are approximately 40 people diagnosed each year with this specific type of brain tumor.
Who Is Diagnosed with Gliomatosis Cerebri?
Gliomatosis cerebri will usually occur more often in people between the ages of 46 and 53. However, the disease can occur at any age range.
Gliomatosis cerebri occurs in both genders, however, it usually presents slightly more frequently in males than in females.
Does Gliomatosis Cerebri Spread?
Gliomatosis cerebri spreads quickly through other areas of the Central Nervous System and cerebrospinal fluid. This tumor is able to spread deeply into brain tissue however it is unable to spread outside of the central nervous system.
White matter is where glial cells are located and the glial cells are the ones in which gliomatosis cerebri tumors grow.
Glioblastoma multiforme is closely related to gliomatosis cerebri and it progresses rapidly. Glioblastoma multiforme is a grade IV tumor and the progression is much faster than those of grades II and III.
While Gliomatosis cerebri has the possibility of being in grades II and III, glioblastoma is always a grade IV tumor.
Due to being the highest grade tumor with rapid growth, the median survival rate of patients with this tumor is 15-16 months. This includes those who have had surgery, radiation and chemotherapy.
Those with lower gliomatosis cerebri may survive longer than those with the highest grade tumor. The progression of gliomatosis cerebri simply depends on the severity and stage of the tumor.
Diagnosis of Gliomatosis Cerebri
Gliomatosis cerebri pathology is not considered in the diagnosis of this type of tumor. It is based strictly on imaging unlike other types of tumors, which are diagnosed using a mixture of both and or solely rely on pathology.
The diagnosis of gliomatosis cerebri can be very complicated because it is unlike other tumors. Its diffuse, threadlike appearance with no clear borders can make it hard to diagnose right away.
Both MRI and CT scans may be used to diagnose a gliomatosis cerebri tumor. These scans can create detailed images of the brain and spine.
In most other tumors, these detailed images would easily detect the presence of a solid tumor. However, with the unique pattern of a gliomatosis cerebri tumor, it may go undetected even with advanced imaging.
A surgical biopsy can help doctors form a comprehensive diagnosis of gliomatosis. During a surgical biopsy, the physician will take a small sample of the tumor to test in a laboratory.
This test will reveal the nature of the tumor and can help physicians understand the best treatment route for each patient.
What Do Gliomatosis Cerebri Tumors Look Like on an MRI?
Gliomatosis cerebri usually appears as abnormal tissue in three or more of the lobes in the brain. There are two different ways that gliomatosis cerebri shows up on an MRI.
- Type 1 does not show an obvious mass. However, it shows a widespread tumor pattern. It is thought to look “fluffy” in appearance, which is abnormal for the brain.
- Type 2 does show a widespread fluffy looking pattern of the tumor. This is similar to the first type. However, type 2 also shows a distinct tumor mass.
The fluffy appearance of the tumor is a characteristic of all gliomatosis cerebri cases.
Treatment and Care Options for Gliomatosis Cerebri 5
There are a variety of treatment options for gliomatosis cerebri. Unfortunately, there is no cure for this specific type of tumor.
It is possible for the tumor to be removed or shrunk in order to relieve pressure on the brain and to attempt to eliminate the tumor.
While it can relieve pressure, due to the rapid growth of the tumor, surgery is unlikely to completely remove every part of the tumor. Since this type of tumor is also very diffuse in appearance, it makes it hard to remove each aspect of the tumor.
Another use of surgery is to place a shunt replacement. Some tumors in the brain may block the normal flow of cerebrospinal fluid. This blockage can cause hydrocephalus which is a build-up of fluid pressure in the brain.
Temporary drains can be placed to relieve pressure, however, a shunt is a more permanent solution to the blockage.
This makes allows the cerebrospinal fluid to drain into the patient's abdomen to be absorbed into the body to prevent accumulation in the brain.
Chemotherapy is used in an attempt to kill any cancer cells that may have been left behind following surgery. Chemotherapy can be used to help shrink the tumor as it targets to kill any type of cell in the body that reproduces rapidly.
The downside of chemotherapy is that it is impossible to target cancer cells only, so it always works systemically. This means that the whole body is affected, not just the site of the tumor.
This is why there are so many side effects that follow the use of chemotherapy. These side effects are typically caused by the death of healthy fast-growing cells in the body.
The cells that are most likely to be damaged by chemotherapy are blood-forming cells, hair follicles and cells in the mouth, digestive tract and reproductive system.
The cells listed above are the fastest-growing healthy cells in the body and the death of these can lead to some serious side effects.
Death of blood-forming cells in bone marrow, for example, means that there will be a decrease in white blood cells which are responsible for helping the body fight infection.
This means that patients will be more susceptible to infections because the body won’t be able to fight it off during chemotherapy.
Another complication is that there will be a decrease in red blood cells. Red blood cells are responsible for carrying oxygen. With decreased red blood cells, anemia can occur and the body will feel fatigued and possibly short of breath.
Lastly, a decrease in blood-forming cells means that there will be a decrease in platelets in the body. Platelets are responsible for helping the blood to clot to stop bleeding.
With a decrease in platelets, the body will be unable to stop itself from bleeding excessively.
Chemotherapy can be given via a pill, injection or IV drip in the hospital.
Radiation therapy can be used in addition to or instead of surgery in order to help control the growth of the tumor. Radiation uses beams of intense energy to target and destroy cancer cells.
Gliomatosis cerebri radiation is used to target a specific area of the body. Unfortunately, radiation still kills both healthy and cancerous cells.
This means that the same side effects that occur with chemotherapy, can occur with radiation as well.
There are various clinical trials in the field right now to help researchers understand and find more effective ways to treat this disease 6. One clinical trial that is available currently is to test the usage of nivolumab in patients with gliomatosis cerebri. Participants will receive this drug intravenously every 2 weeks for up to 64 weeks and will be monitored for up to 5 years to examine the effects.
How Are Side Effects Managed?
There are other treatments to manage the complications that can arise in patients with this type of tumor. As stated previously, seizures and swelling are common side effects of gliomatosis cerebri tumors.
So steroid treatments to reduce swelling and anti-seizure medications to prevent seizures may also be used in conjunction with other options to enhance the quality of life of patients with this tumor.
Prognosis for Gliomatosis Cerebri
The long-term outlook and survival rates for gliomatosis cerebri are very poor.
Not enough research has been done to establish an effective cure for this disease and due to the rapid growth of the tumors, most patients do not survive for longer than two years.
The five-year survival rate for patients with gliomatosis cerebri is 18.8%.
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- Gliomatosis cerebri - Diagnosing and Treating Gliomatosis Cerebri.
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- Immune Checkpoint Inhibitor Nivolumab in People With Select Rare CNS Cancers.
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