Glioma refers to a type of brain tumor that develops from the glial cells, which are specialized cells that surround and support neurons (nerve cells) in the brain. It is generally classified based on which type of glial cell is involved in the tumor:

• Astocytoma - tumors that develop from star-shaped glial cells called astrocytes
• Ependymomas - tumors that arise from ependymal cells that line the ventricles of the brain and the center of the spinal cord
• Oligodendrogliomas - tumors that affect the oligodendrocytes

The symptoms of glioma vary by type but may include headaches; nausea and vomiting; confusion; personality changes; trouble with balance; vision problems; speech difficulties; and/or seizures. The exact underlying cause is unknown. In most cases, the tumor occurs sporadically in people with no family history of the condition. Treatment depends on many factors, including the type, size, stage and location of the tumor, but may include surgery, radiation therapy, chemotherapy and/or targeted therapy.

Anaplastic ependymoma is a type of ependymoma, which is a tumor that forms when cells in the central nervous system (including the brain and spinal cord) begin to multiply rapidly. An ependymoma is anaplastic if the cells grow very quickly and are significantly unusual in shape. Ependymomas can occur at any age. When ependymomas occur in children, they are more frequently located in the brain (intracranial). Ependymomas are more often found in the spinal cord of affected adults. Symptoms of an anaplastic ependymoma depend on the age of the person and the size and location of the tumor but may include headaches, nausea, vision changes, seizures, and changes in mood or personality.

The exact cause of an anaplastic ependymoma is not known, but it is thought that certain changes (acquired pathogenic variants, also known as somatic mutations ) in specific genes in some cells of the body allow the cells to begin growing quickly. A diagnosis of anaplastic ependymoma is generally suspected based on imaging with MRI or CT scan. The diagnosis can be confirmed with a biopsy of the tumor or when the tumor is removed (resected). Treatment for anaplastic ependymoma begins with surgery to remove as much of the tumor as possible. Radiation therapy is also recommended for people with anaplastic ependymoma.

An ependymoma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of ependymomas?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Ependymomas are grouped in three grades based on their characteristics (grade 1, 2, or 3, also written as grade I, II, or III). Within each grade, are different ependymoma subtypes. Molecular testing is used to help identify subtypes that are related to location and disease characteristics.

1. Grade 1 ependymomas are low grade tumors. This means the tumor cells grow slowly. The subtypes include subependymoma and myxopapillary ependymoma. Both are more common in adults than in children. Myxopapillary tumors usually occur in the spine.
2. Grade 2 ependymomas are low grade tumors and can occur in either the brain or the spine.
3. Grade 3 ependymomas are malignant (cancerous). This means they are fast-growing tumors. The subtypes include anaplastic ependymomas. These most often occur in the brain, but can also occur in the spine.

What do ependymomas look like on an MRI?

Ependymomas usually appear as a well-defined mass that often brightens with contrast.

What causes ependymomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of ependymomas is not known.

Where do ependymomas form?

Ependymomas can form anywhere in the CNS. Ependymomas often occur near the ventricles in the brain and the central canal of the spinal cord. On rare occasions, ependymomas can form outside the CNS, such as in the ovaries. Ependymomas develop from ependymal cells (called radial glial cells). Ependymal cells are one of three types of glial cells that support the CNS.

Do ependymomas spread?

Ependymomas rarely spread outside the CNS. But ependymomas can spread to other areas of the CNS through cerebrospinal fluid (CSF).

What are symptoms of an ependymoma?

Symptoms related to an ependymoma depend on the tumor’s location. Here are some possible symptoms that can occur.

Ependymoma Symptoms

People with an ependymoma in the brain may have:

• Headaches
• Nausea
• Vomiting
• Dizziness

People with an ependymoma in the spine may have:

• Back pain
• Numbness and weakness in their arms, legs or trunk
• Problems with sexual, and urinary or bowel problems

Who is diagnosed with ependymomas?

Ependymomas occur in both children and adults. Ependymomas in the lower half of the brain are more common among children. Ependymomas in the spine are more common among adults. Ependymomas occur more often in males than females. They are most common in white and non-hispanic people. An estimated 13,294 people are living with this tumor in the United States.

What is the prognosis of ependymomas?

The likely outcome of the disease or chance of recovery is called prognosis.

Ependymoma Prognosis

The relative 5-year survival rate for ependymoma is 83.9% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are treatment options for ependymomas?

The first treatment for an ependymoma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.

After surgery, there is no standard treatment for ependymomas. Many people won’t need other treatment after surgery. For other people, treatments may include radiation, chemotherapy or clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

There are several different types of glia with different functions, two of which are shown in Figure 35.7. Astrocytes, shown in Figure 35.8a make contact with both capillaries and neurons in the CNS. They provide nutrients and other substances to neurons, regulate the concentrations of ions and chemicals in the extracellular fluid, and provide structural support for synapses. Astrocytes also form the blood-brain barrier—a structure that blocks entrance of toxic substances into the brain. Astrocytes, in particular, have been shown through calcium imaging experiments to become active in response to nerve activity, transmit calcium waves between astrocytes, and modulate the activity of surrounding synapses. Satellite glia provide nutrients and structural support for neurons in the PNS. Microglia scavenge and degrade dead cells and protect the brain from invading microorganisms. Oligodendrocytes, shown in Figure 35.8b form myelin sheaths around axons in the CNS. One axon can be myelinated by several oligodendrocytes, and one oligodendrocyte can provide myelin for multiple neurons. This is distinctive from the PNS where a single Schwann cell provides myelin for only one axon as the entire Schwann cell surrounds the axon. Radial glia serve as scaffolds for developing neurons as they migrate to their end destinations. Ependymal cells line fluid-filled ventricles of the brain and the central canal of the spinal cord. They are involved in the production of cerebrospinal fluid, which serves as a cushion for the brain, moves the fluid between the spinal cord and the brain, and is a component for the choroid plexus.

Figure 35.7 Glial cells support neurons and maintain their environment. Glial cells of the (a) central nervous system include oligodendrocytes, astrocytes, ependymal cells, and microglial cells. Oligodendrocytes form the myelin sheath around axons. Astrocytes provide nutrients to neurons, maintain their extracellular environment, and provide structural support. Microglia scavenge pathogens and dead cells. Ependymal cells produce cerebrospinal fluid that cushions the neurons. Glial cells of the (b) peripheral nervous system include Schwann cells, which form the myelin sheath, and satellite cells, which provide nutrients and structural support to neurons.

Figure 35.8 (a) Astrocytes and (b) oligodendrocytes are glial cells of the central nervous system. (credit a: modification of work by Uniformed Services University; credit b: modification of work by Jurjen Broeke; scale-bar data from Matt Russell)

Neurogenesis

At one time, scientists believed that people were born with all the neurons they would ever have. Research performed during the last few decades indicates that neurogenesis, the birth of new neurons, continues into adulthood. Neurogenesis was first discovered in songbirds that produce new neurons while learning songs. For mammals, new neurons also play an important role in learning: about 1000 new neurons develop in the hippocampus (a brain structure involved in learning and memory) each day. While most of the new neurons will die, researchers found that an increase in the number of surviving new neurons in the hippocampus correlated with how well rats learned a new task. Interestingly, both exercise and some antidepressant medications also promote neurogenesis in the hippocampus. Stress has the opposite effect. While neurogenesis is quite limited compared to regeneration in other tissues, research in this area may lead to new treatments for disorders such as Alzheimer’s, stroke, and epilepsy.

How do scientists identify new neurons? A researcher can inject a compound called bromodeoxyuridine (BrdU) into the brain of an animal. While all cells will be exposed to BrdU, BrdU will only be incorporated into the DNA of newly generated cells that are in S phase. A technique called immunohistochemistry can be used to attach a fluorescent label to the incorporated BrdU, and a researcher can use fluorescent microscopy to visualize the presence of BrdU, and thus new neurons, in brain tissue. Figure 35.6 is a micrograph which shows fluorescently labeled neurons in the hippocampus of a rat.

Figure 35.6 This micrograph shows fluorescently labeled new neurons in a rat hippocampus. Cells that are actively dividing have bromodeoxyuridine (BrdU) incorporated into their DNA and are labeled in red. Cells that express glial fibrillary acidic protein (GFAP) are labeled in green. Astrocytes, but not neurons, express GFAP. Thus, cells that are labeled both red and green are actively dividing astrocytes, whereas cells labeled red only are actively dividing neurons. (credit: modification of work by Dr. Maryam Faiz, et. al., University of Barcelona; scale-bar data from Matt Russell)

Diffuse midline gliomas are primary central nervous system (CNS) tumors. This means they begin in the brain or spinal cord. Diffuse midline glioma is a rare subtype of glial tumors.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of diffuse midline gliomas?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Diffuse midline gliomas are all grade 4 tumors (also written as grade IV). This means they are malignant (cancerous) and fast-growing.

What do diffuse midline gliomas look like on an MRI?

Diffuse midline gliomas usually appear as a mass that has spread in the middle area of the brain. The tumor shows degrees of brightness with contrast. In adults, the diagnosis of diffuse middling glioma can’t be made by imaging alone because the tumors look similar to glioblastomas and other high-grade brain cancers.

What causes diffuse midline gliomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of diffuse midline gliomas is not known. People with gene changes that can be passed down through families, such as Li-Fraumeni syndrome and neurofibromatosis type I, are at increased risk for developing a diffuse midline glioma. Changes in a gene known as the h3K27M mutation, have also been linked to the growth of diffuse midline glioma.

Where do diffuse midline gliomas form?

Diffuse midline glioma most often forms in the pons in the brainstem, thalamus, spinal cord, and cerebellum. It’s uncommon for these tumors to occur in other areas of the CNS. The tumors are named, in part, based on the locations where these tumors most often occur. Therefore, tumors in other brain or spine locations thought to be diffuse midline glioma should be reviewed by neuro-oncology providers with experience in treating people with these tumors.

Diffuse midline glioma is a rare type of astrocytoma that look similar under the microscope to other more common astrocytomas, such as a glioblastoma. Since it can be hard to tell them apart, they require molecular testing. Some diffuse midline gliomas have changes in histone-related genes, the most common is h3K27M. Review by a neuropathologist is recommended to confirm this diagnosis.

Do diffuse midline gliomas spread?

Diffuse midline gliomas are fast-growing tumors and can spread to other areas of the CNS through cerebrospinal fluid (CSF). The tumor can invade nearby tissue and extend beyond what can be seen on MRI or by the surgeon during surgery. Diffuse midline glioma, however, usually grow and come back in the same location in which they first appeared.

What are the symptoms of diffuse midline gliomas?

Symptoms related to diffuse midline glioma depend on the tumor’s location. Here are some possible symptoms that can occur.

Diffuse Midline Glioma Symptoms

• Double vision
• Problems swallowing
• Weakness on one or both sides of the body
• Loss of balance

Diffuse midline glioma in the brain also may cause increased pressure within the skull due to production of too much CSF or blockage of its normal flow. This problem is known as hydrocephalus.

Signs and symptoms of hydrocephalus may include:

• Nausea
• Vomiting
• Irritability
• Headaches
• Blurred or double vision
• A strong desire to sleep
• Seizures

People with a diffuse midline glioma in the spine may have:

• Progressive weakness
• Numbness
• Problems with bowel and bladder control

Who is diagnosed with diffuse midline gliomas?

Diffuse midline gliomas occur in both children and adults. Diffuse midline glioma tends to occur in children. Diffuse midline gliomas occur more often in males than females. They are most common in white and non-hispanic people. An estimated 6,033 people are living with this tumor in the United States.

What is the prognosis of diffuse midline gliomas?

The likely outcome of the disease or chance of recovery is called prognosis.

Diffuse Midline Glioma Prognosis

The relative 5-year survival rate for diffuse midline glioma is 48.9% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are the treatment options for diffuse midline gliomas?

The first treatment for diffuse midline glioma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person. The brainstem, thalamus, and spinal cord are sensitive locations in the CNS and surgery in these areas can cause serious loss of function. Diffuse midline glioma may sometimes be treated with radiation therapy alone without a biopsy if the risk of biopsy is thought to be too high. A procedure such as a ventriculoperitoneal shunt, may also be needed to redirect the flow of cerebrospinal fluid if the patient has hydrocephalus.

Diffuse midline gliomas usually require further treatments. Treatments may include radiation, chemotherapy, or clinical trials. Surgery is followed by radiation therapy in most cases. Chemotherapy is sometimes given with radiation therapy, or after radiation. However, there is no standard chemotherapy regimen, and each plan should be tailored to each patient. Review of diffuse midline glioma by a team of experts including neuro-oncologists, neuroradiologists, radiation oncologists, and neurosurgeons is important to determine the best treatment plan. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option.

Gliomatosis cerebri is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. This tumor is no longer recognized as a formal diagnosis, rather gliomatosis cerebri refers to a diffuse pattern of glioma cells with extensive growth that invade multiple lobes of the brain. Gliomas of different grade and cell of origin (astrocytes, oligodendrocytes) can grow with this pattern, and very little is understood about the molecular basis of the disease. More research is needed to discover the origin of these tumors and to improve their treatment.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of gliomatosis cerebri?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Gliomatosis cerebri are grouped in three grades based on their characteristics (grade 2, 3, or 4 also written as grade II, III, or IV).

1. Grade 2 gliomatosis cerebri are mid-grade tumors. This means the tumors have a higher chance of coming back after being removed. They usually have a change in the genes called isocitrate dehydrogenase (IDH).
2. Grade 3 and 4 gliomatosis cerebri are malignant (cancerous). This means they are fast-growing tumors that often become resistant to treatment.

What do gliomatosis cerebri look like on an MRI?

Gliomatosis cerebri usually appear abnormal in three or more lobes of the brain.

1. Type 1 shows no obvious mass, but a widespread tumor pattern or a fluffy looking abnormality.
2. Type 2 shows a widespread fluffy looking tumor pattern, but also a tumor mass.

Gliomatosis cerebri is diagnosed based on imaging, instead of pathology like most other tumor types.

What causes gliomatosis cerebri?

The cause of gliomatosis cerebri are not known.

Where does gliomatosis cerebri form?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. Gliomatosis cerebri commonly arises from glial cells in the brain. Glial cells support and insulate neurons and are the most abundant cells in the brain. When seen under a microscope the tumor cells resemble glial cells and involve many areas of the cerebrum. This is why this is named gliomatosis cerebri.

Do gliomatosis cerebri spread?

Gliomatosis cerebri can spread to other areas of the CNS through cerebrospinal fluid (CSF). They can spread quickly and deeply into surrounding brain tissue. They do not spread outside the CNS.

What are symptoms of gliomatosis cerebri?

Symptoms related to a gliomatosis cerebri depend on the tumor’s location and person’s age. Here are some possible symptoms that can occur.

Gliomatosis Cerebri Symptoms

• Seizures
• Fatigue
• Mood changes
• Changes in thinking and memory
• Headaches

Who is diagnosed with gliomatosis cerebri?

Gliomatosis cerebri occur most often in people between the ages of 46 and 53, but can occur at any age. Gliomatosis cerebri occur slightly more often in males than females. An estimated 247 people are living with this tumor in the United States.

What is the prognosis of gliomatosis cerebri?

The likely outcome of the disease or chance of recovery is called prognosis.

Gliomatosis Cerebri Prognosis

The relative 5-year survival rate for gliomatosis cerebri is 18.8% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are treatment options for gliomatosis cerebri?

The first treatment for a gliomatosis cerebri is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and remove as much of the tumor as possible without causing more symptoms for the person. Surgery is usually limited to a biopsy, as there is not a central mass for removal.

After surgery, there is no standard treatment for gliomatosis cerebris. Other treatments may include radiation, chemotherapy, or clinical trials. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location. This tumor type may respond well to radiation therapy, but patients should discuss the full risks and benefits with their healthcare team, as radiation to a large volume of brain tissue puts normal brain tissue at risk for problems. Chemotherapy is also a treatment that may be recommended during radiation, after radiation is completed, or if the tumor recurs after initial treatment. Temozolomide is a drug that is often used, as this is the therapy for astrocytic type tumors. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option.

Gliosarcoma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. Gliosarcoma is a rare type of glioma.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of gliosarcomas?

CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Gliosarcomas are all classified as grade 4 (also written as grade IV) tumors. This means they are malignant (cancerous) and fast-growing. They are further described as primary or secondary:

• Primary: The tumor is diagnosed from the first surgery or biopsy.
• Secondary: The tumor forms from a pre-existing glioma, usually after radiation treatment.

What do gliosarcomas look like on an MRI?

Gliosarcomas usually appear as a single tumor with vague borders. The tumors may occur near the surface of the brain and be attached to the covering of the brain. The tumor often brightens with contrast and many tumors can occur in different areas of the CNS. Gliosarcomas usually have some swelling around them.

What causes gliosarcomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of most gliosarcomas is not known. Previous exposure to radiation and certain gene changes that can be passed down through families have been linked to a higher chance of developing glioblastomas.

Where do gliosarcomas form?

Gliosarcomas are commonly found along the surface of the brain, but can form in the spinal cord. They arise from glial cells, the supportive cells in the brain. Gliosarcomas develop with two different characteristics. Some resemble astrocytes, a star-shaped glial cell and the most abundant cell in the brain. Other gliosarcomas resemble connective tissue cells called sarcomas.

Do gliosarcomas spread?

Gliosarcomas can spread to other areas of the CNS through cerebrospinal fluid (CSF). Gliosarcomas generally don’t spread outside of the CNS to other organs.

What are the symptoms of gliosarcomas?

Symptoms related to gliosarcomas depend on the tumor’s location. Here are some possible symptoms that can occur.

Gliosarcoma Symptoms

• Headaches
• Seizures
• Thinking or memory difficulties
• Weakness
• Numbness
• Problems with balance and movement

Who is diagnosed with gliosarcomas?

Gliosarcomas occur most often in people between the ages of 40 and 60, but can occur at any age. Gliosarcomas occur slightly more often in males than females. They are most common in white and non-hispanic people. An estimated 557 people are living with this tumor in the United States.

What is the prognosis of gliosarcomas?

The likely outcome of the disease or chance of recovery is called prognosis.

Gliosarcoma Prognosis

The relative 5-year survival rate for gliosarcoma is 5.6% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are the treatment options for gliosarcomas?

The first treatment for a gliosarcoma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.

Gliosarcomas are aggressive so most people receive further treatments. Treatments may include radiation, chemotherapy, or taking part in clinical trials. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

Medulloblastoma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of medulloblastomas?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Medulloblastomas are all classified as grade 4 (also written as grade IV) tumors. This means they are malignant (cancerous) and fast-growing.

There are four subtypes that have been identified in children with medulloblastoma.

1. WNT-activated
2. SHH-activated
3. Group 3 (non-WNT / non-SHH)
4. Group 4 (non-WNT / non-SHH)

In adults with medulloblastoma, these subtypes are not as well-defined as they are in children.

What does it look like on an MRI?

Medulloblastomas usually appear as a solid mass in the cerebellum, which is the portion of the brain in the back of the head between the cerebrum and the brain stem. The tumor often brightens with contrast. Once the mass is known to be a medulloblastoma, a MRI of the spinal cord is recommended to see if it has spread.

What causes medulloblastomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of most medulloblastomas is not known. There’s a small percent of childhood medulloblastomas that are related to gene changes that can be passed down through families. There’s no known cause for adults diagnosed with medulloblastomas.

Where do medulloblastomas form?

Medulloblastomas most commonly form in the cerebellum, the bottom part of the brain located at the back of the skull. Medulloblastoma is known as an embryonal neuroepithelial tumor because it forms in fetal cells that remain after birth.

Do medulloblastomas spread?

Medulloblastomas are very fast-growing. They often spread to other areas of the CNS through cerebrospinal fluid (CSF). Rarely, medulloblastomas can spread outside the CNS to the bones or the lymphatic system.

What are symptoms of a medulloblastoma?

Symptoms related to a medulloblastoma depend on the tumor’s location. Here are some possible symptoms that can occur.

Medulloblastoma Symptoms

People with a medulloblastoma in the cerebellum may have:

• Issues with walking, balance, and/or fine motor skills

If the tumor is blocking the CSF, this can lead to increased pressure inside the skull. This problem is known as hydrocephalus.

Signs and symptoms of hydrocephalus may include:

• Headaches
• Nausea
• Vomiting
• Blurred and double vision
• Extreme sleepiness
• Confusion
• Seizures and even passing out

If medulloblastoma has spread to the spine, symptoms may include:

• Weakness or numbness in the arms and or legs
• A change in normal bowel or bladder habits
• Spinal pain

Who is diagnosed with medulloblastoma?

Medulloblastoma can occur in both children and adults, but is more common in children. In adults, medulloblastoma typically affects younger adults between 20 and 40 years of age. Medulloblastomas occur more often in males than females. They are most common in white and hispanic people. An estimated 3,840 people are living with this tumor in the United States.

What is the prognosis of medulloblastoma?

The likely outcome of the disease or chance of recovery is called prognosis.

Medulloblastoma Prognosis

The relative 5-year survival rate for medulloblastoma is 72.1% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are treatment options for medulloblastoma?

The first treatment for medulloblastoma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person.

Most people with medulloblastomas receive further treatments. Treatments may include radiation, chemotherapy, or taking part in clinical trials. Surgery is usually followed by radiation to the brain and spine since this disease tends to spread in the brain and spine. Because of problems with bone marrow and surrounding organs that occur with brain and spine radiation, proton beam radiation therapy is often recommended. Chemotherapy may also be part of the treatment plan, depending on the subtype and amount the tumor has spread. Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. It is important to seek expert recommendations from a neuro-oncologist with experience in adult medulloblastoma. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type and tumor location.

Oligodendroglioma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. Diagnosing oligodendroglioma requires finding two genetic alterations: an IDH mutation and a very specific change in the tumor cell’s chromosomes where the short arm of chromosome 1 and the long arm of chromosome 19 is lost; also known as 1p19q codeletion. Content on other IDH-mutated tumors will be coming soon.

To get an accurate diagnosis, a piece of tumor tissue will be removed during surgery, if possible. A neuropathologist should then review the tumor tissue.

What are the grades of oligodendrogliomas?

Primary CNS tumors are graded based on the tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery, if surgery is possible.

Oligodendrogliomas are grouped in two grades (grade 2 or grade 3, also written as grade II or grade III) based on their characteristics.

1. Grade 2 oligodendrogliomas are low grade tumors. This means the tumor cells grow slowly and invade nearby normal tissue. In many cases, they form years before being diagnosed as no symptoms appear.
2. Grade 3 oligodendrogliomas are malignant (cancerous). This means they are fast-growing tumors. They are called anaplastic oligodendriogliomas.

What do oligodendrogliomas look like on an MRI?

Oligodendrogliomas usually appear as a single tumor with well-defined borders. The tumor may enhance with contrast and is most often seen in anaplastic oligodendrogliomas. Oligodendrogliomas tend to have some swelling around them.

What causes oligodendrogliomas?

Cancer is a genetic disease – that is, cancer is caused by certain changes to genes that control the way our cells function. Genes may be mutated (changed) in many types of cancer, which can increase the growth and spread of cancer cells. The cause of most oligodendrogliomas is not known. Exposure to radiation and certain gene changes that can be passed down through families have been linked to a higher chance of developing oligodendrogliomas.

Where do oligodendrogliomas form?

Oligodendrogliomas are commonly found in the white matter and the outer layer of the brain, called the cortex, but can form anywhere in the CNS. These tumors are called oligodendrogliomas because the cells resemble oligodendrocytes, a type of brain cell that supports and insulates nerve fibers in the CNS.

Do oligodendrogliomas spread?

Oligodendrogliomas can spread to other areas of the CNS through cerebrospinal fluid (CSF), but is uncommon. Oligodendrogliomas rarely spread outside of the CNS to other organs.

What are the symptoms of an oligodendroglioma?

Symptoms related to oligodendrogliomas depend on the tumor’s location. Here are some possible symptoms that can occur.

Oligodendroglioma Symptoms

The most common sign of an oligodendroglioma is a seizure. Around 60% of people have a seizure before being diagnosed.

Other symptoms people may have:

• Headaches
• Problems with thinking and memory
• Weakness
• Numbness
• Problems with balance and movement

Who is diagnosed with oligodendrogliomas?

Oligodendrogliomas occur most often in people between the ages of 35 and 44, but can occur at any age. Oligodendrogliomas occur more often in males and are rare in children. They are most common in white and non-hispanic people. An estimated 11,757 people are living with this tumor in the United States.

What is the prognosis of oligodendrogliomas?

The likely outcome of the disease or chance of recovery is called prognosis.

Oligodendroglioma Prognosis

The relative 5-year survival rate for oligodendroglioma is 74.1% but know that many factors can affect prognosis. This includes the tumor grade and type, traits of the cancer, the person’s age and health when diagnosed, and how they respond to treatment. If you want to understand your prognosis, talk to your doctor.

What are the treatment options for oligodendrogliomas?

The first treatment for an oligodendroglioma is surgery, if possible. The goal of surgery is to obtain tissue to determine the tumor type and to remove as much tumor as possible without causing more symptoms for the person. Treatments after surgery may include radiation, chemotherapy, or clinical trials.

Clinical trials, with new chemotherapy, targeted therapy, or immunotherapy drugs, may also be available and can be a possible treatment option. Treatments are decided by the patient’s healthcare team based on the patient’s age, remaining tumor after surgery, tumor type, and tumor location.

Your oncologist might recommend a combination of medications - procarbazine, lomustine, and vincristine. A chemotherapy called temozolomide is also being studied in clinical trials.

Glial cell derived tumors arising from the optic nerve, usually presenting in childhood.

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

• 5-aminolevulinic acid(Brand name: Gleolan)
  FDA-approved indication: Optical imaging agent indicated in patients with glioma (suspected World Health Organization Grades III or IV on preoperative imaging) as an adjunct for the visualization of malignant tissue during surgery.
• Polifeprosan 20 with carmustine(Brand name: Gliadel®)
  FDA-approved indication: Expanding the indication to include patients with malignant glioma undergoing primary surgical resection.
• Temozolomide(Brand name: Temodar®)
  FDA-approved indication: Treatment of adult patients with newly diagnosed glioblastoma multiforme concomitatly with radiotherapy and then as maintenance treatment

Myxopapillary ependymoma (MEPN) is a slow-growing ependymoma (a type of glioma, which is a tumor that arises from the supportive tissue of the brain and spinal cord). They tend to occur in the lower part of the spinal column and are usually considered to be benign, low-grade or grade I tumors. The age of diagnosis ranges from 6 to 82 years. Symptoms of an ependymoma are related to the location and size of the tumor and may include nausea, vomiting, headache, pain, numbness, bowel or bladder symptoms, and various other signs and symptoms. The cause of ependymomas is unknown. They are known to recur locally (more commonly in individuals diagnosed in childhood). Treatment may vary depending on the location, grade, and whether the tumor has spread to the spine, but typically includes aggressive surgery. Management may also include chemotherapy and radiation therapy.

Other types of tumors can form in glial cells and nerve cells. Neuronal tumors are rare tumors made up of nerve cells. Glioneuronal tumors are a mix of nerve cells and glial cells. Neuronal and glioneuronal tumors are rare, low-grade tumors and are treated the same as gliomas.

• Low-grade gliomas grow slowly and do not spread within the brain and spinal cord. But as they grow, they press on nearby healthy areas of the brain, affecting brain function. Most low-grade gliomas are treatable.
• Sometimes a low-grade glioma can come back as a high-grade glioma. High-grade gliomas often come back within 3 years either in the place where the cancer first formed or somewhere else in the brain or spinal cord.

Treatment of newly diagnosed childhood low-grade glioma, astrocytoma, neuronal, and glioneuronal tumors

Children with neurofibromatosis type 1 and a central nervous system tumor, children with an optic pathway glioma, or children who had a tumor found when getting a scan for another health problem may be observed (closely watched). These children may not receive treatment until signs or symptoms appear or change or the tumor grows.

Children with tuberous sclerosis may develop low-grade tumors in the brain called subependymal giant cell astrocytoma (SEGAs). Targeted therapy with everolimus or sirolimus may be used instead of surgery, to shrink the tumors.

Children diagnosed with low-grade glioma are treated based on where the tumor is located. The first treatment is usually surgery. An MRI is done after surgery to see if any tumor remains. If the tumor was completely removed by surgery, more treatment may not be needed and the child is closely observed.

If there is tumor remaining after surgery, treatment may include:

• observation for children who had surgery to remove part of the tumor and the tumor is expected to regrow slowly
• combination chemotherapy with or without radiation therapy
• radiation therapy, which may include conformal radiation therapy, intensity-modulated radiation therapy, proton beam radiation therapy, or stereotactic radiation therapy, may be used if the tumor does not respond to chemotherapy or when the tumor begins to grow again
• targeted therapy (trametinib and dabrafenib)
• a clinical trial of targeted therapy (selumetinib) with or without chemotherapy