Who treats central nervous system (CNS) tumors?

People with CNS tumors are usually cared for by a team of healthcare professionals who have special training. In most cases, and depending on your needs, the following providers will be part of your care team: 

• Neuro-oncologist: A doctor who specializes in the diagnoses and treatment of CNS tumors and other neurologic complications of cancer.
• Oncologist: A doctor who treats cancer, including CNS tumors.
• Neurologist: A doctor who manages disorders of the CNS.
• Neurosurgeon: A doctor who diagnoses and surgically manages CNS diseases.
• Radiation Oncologist: A doctor who plans and gives radiation therapy.
• Neuroradiologist: A doctor who diagnoses diseases of the CNS using imaging techniques such as MRI, CT scan, and PET scan.
• Neuropathologist: A doctor who examines CNS tissue to determine diagnosis.
• Neuropsychologist: A psychologist who studies the function and structure of the brain and the thinking, memory, and behavior changes that can result from problems in the brain.
• Psychologist: A healthcare professional who evaluates both normal and abnormal mental states and behavior.
• Social Worker: A healthcare professional who helps people cope and resolve problems in their everyday lives by providing guidance and resources.
• Genetic Counselor: A healthcare professional who evaluates risk and management of inherited conditions for people and families.
• Nurse Practitioner and Physician Assistant: A healthcare professional who diagnoses and manages acute and chronic illnesses.
• Nurse: A healthcare professional who cares for people, families, and communities, and oversees the management of care as prescribed by a doctor, nurse practitioner, or physician assistant. Nurses focus on inpatient care, outpatient care, hospice care, or management of research studies, as examples. 

People with a rare CNS tumor should seek a medical diagnosis and disease care and treatment with a team experienced in their disease.

How are tumors monitored?

People with CNS tumors are usually monitored with imaging procedures. The most common are magnetic resonance imaging (MRI), which may include a specialized MRI such as spectroscopy. Sometimes computed tomography (CT) scans and positron emission topography (PET) are also used. How often you have imaging studies will be decided by your physician. A neuroradiologist will read your results from your scans and share them with your physician.

Who reviews tumors to determine an accurate diagnosis?

A neuropathologist should review your tumor tissue. The neuropathologist will determine the grade and type of tumor based on how it looks under the microscope.  He or she will also describe your tumor based on genetic changes and share the information with your physician.

How are tumors graded?

Primary CNS tumors are grouped by how the tumor tissue looks under a microscope and, sometimes, by certain genetic changes that may be present in the tumor. CNS tumors are graded based on World Health Organization (WHO) criteria as grade 1, grade 2, grade 3 or grade 4 (also written as grade I, II, III, or IV).  Some tumors are low grade tumors (grade 1 and grade 2) and others are high grade (grade 3 and 4).  Treatment and prognosis of primary CNS tumors depends on tumor location, tumor type, extent of tumor spread, genetic findings, the patient’s age, and tumor remaining after surgery. 

• Grade 1: The tumor cells look more like normal cells under a microscope and grow and spread more slowly than grade II, III, and IV tumor cells. They rarely spread into nearby tissues. Grade I CNS tumors may be cured if they’re completely removed by surgery.
• Grade 2: The tumor cells grow and spread more slowly than grade III and IV tumor cells. They may spread into nearby tissue and may come back as a higher-grade tumor.
• Grade 3: The tumor cells look very different from normal cells under a microscope and grow more quickly than grade I and II tumor cells. They’re likely to spread into nearby tissue.
• Grade 4 (high-grade): The tumor cells don’t look like normal cells under a microscope and grow and spread very quickly.

What is a tumor type?

CNS tumors are grouped together based on either the type of cell they start from or the location in the CNS. Subtypes are then defined within each tumor type, based on specific tissue characteristics or changes in the DNA. The tissue characteristics include:

• How the cancer cells look under a microscope.
• If there are certain substances in or on the cells.
• If there are certain changes to the DNA of the cells.

There are over 100 tumor subtypes. It’s important to know your tumor subtype, if available, in order to plan treatment and determine prognosis. Ask your physician if your tumor has subtypes and how your tissue can be evaluated.

What is prognosis?

You may have questions about how serious your cancer is and your chances of survival. The likely outcome of your disease or your chance of recovery is called prognosis. Many factors can affect your prognosis. This includes your tumor grade and type, traits of your cancer, your age and health when diagnosed, and how you respond to treatment.

Doctors estimate prognosis by using statistics that researchers have collected over many years about people with the same type of cancer. Statistics are based on large groups of people. They cannot be used to predict exactly what will happen to you. If you want to understand your prognosis, talk to your doctor.

Are CNS tumors genetic?

All cancers are genetic diseases. This means they are caused by certain changes in genes that control the way cells function. These changes often happen when cells grow and divide. Most cancers are caused by changes in the genes in the tissue or organ where they develop. Only 5 to 10% of all cancers are hereditary, which means passed down through a family.

Are CNS tumors hereditary?

Most CNS tumors aren’t hereditary. The small percentage of CNS tumors that are hereditary are due to rare syndromes that make someone more likely to have specific cancers. Doctors can find people and families at risk for these conditions by offering genetic counseling and tests. If you are concerned that your family or medical history may increase your chances of developing cancer, talk to a genetic counselor.

The brain controls many important body functions, such as emotions, vision, thought, speech, and movement. The spinal cord connects the brain to nerves in most parts of the body. This allows the brain to send messages throughout the body. The network of the brain and spinal cord is called the central nervous system (CNS). It is important to know your tumor location in the CNS to understand your symptoms and how your tumor may impact body functions.

Brain Anatomy and Functions

The brain is divided down the middle from front to back into two halves called the cerebral hemispheres. Each hemisphere is divided into four lobes: frontal, parietal, occipital, and temporal. Brain tumors located in the lobes are called supratentorial and tumors located in the cerebellum or brainstem are called infratentorial.

Brain tumors, regardless of their location, can cause headaches, seizures and problems with multi-tasking. Learn about specific changes you may experience based on the tumor location below.

Spine Anatomy and Functions

Spinal cord tumors are described based on where on the cord the tumor is located and each vertebra is numbered from top to bottom. The neck region is called cervical (C), the back region is called thoracic (T), and the lower back region is called lumbar (L) or sacral/cauda equina (S). Tumors are further described by whether the tumor begins in the cells inside the spinal cord (intramedullary) or outside the spinal cord (extramedullary). Extramedullary tumors grow in the membrane surrounding the spinal cord (intradural) or outside (extradural).

Spinal cord tumors, regardless of location, often cause pain, numbness, weakness or lack of coordination in the arms and legs, usually on both sides of the body. They also often cause bladder or bowel problems. Learn about specific changes you may experience based on the tumor location below.

Body function changes can be caused by the tumor itself or from treatment. Talk with your healthcare provider about any of these concerns. He or she can assess your problems and suggest options to help manage them.

ATRT 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 is the grade of ATRTs?

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.

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

What do ATRTs look like on an MRI?

ATRTs usually appear very large with fluid-filled areas that often brightens with contrast. You can often see areas of bleeding or dead tissue.

What causes ATRTs?

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. Most ATRTs are caused by changes in a gene known as SMARCB1 (also called INI1). This gene normally signals proteins to stop tumor growth. But in ATRTs, SMARCB1 doesn’t function properly and tumor growth is uncontrolled. SMARCB1 can sometimes be found in a person’s DNA, which means they are born with it.

Where do ATRTs form?

ATRTs can form anywhere in the CNS. They often occur in the brain and often spread to the spinal cord. But ATRTs rarely begin in the spinal cord. ATRTs can form outside the CNS, such as in the kidney, but this is rare. ATRTs develop from several different very young cells called embryonal cells. These cells include rhabdoid, neuroepithelial, epithelial and mesenchymal.

Do ATRTs spread?

ATRTs can be very fast-growing. They often spread to other areas of the CNS through cerebrospinal fluid (CSF).

What are the symptoms of an ATRT?

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

ATRT Symptoms

• Morning headaches
• Vomiting
• Changes in activity levels
• Loss of balance
• Increase in head size (in infants)

Since ATRTs are fast-growing, symptoms usually get worse quickly.

Who is diagnosed with ATRTs?

ATRTs occur in both children and adults and are very rare in both age groups. There have been only 50 reported cases in adults. An estimated 596 people are living with this tumor in the United States. ATRTs occur slightly more often in males than females.

What is the prognosis of ATRTs?

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

ATRT Prognosis

The relative 5-year survival rate for ATRTs is 32.2% 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 ATRTs?

The first treatment for an ATRT 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.

People with ATRTs usually receive further treatments after surgery, which 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.

Choroid plexus tumors are primary central nervous system (CNS) tumors. This means they begin 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 choroid plexus tumors?

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.

Choroid plexus tumors are grouped in three grades based on their characteristics (grade 1, 2, or 3, also written as grade I, II, or III) .

1. Grade 1 choroid plexus papilloma are low grade tumors. This means the tumor cells grow slowly.
2. Grade 2 atypical choroid plexus papilloma are mid-grade tumors. This means the tumors have a higher chance of coming back after being removed.
3. Grade 3 choroid plexus carcinoma are malignant (cancerous). This means they are fast-growing tumors that tend to invade nearby tissue.

What do choroid plexus tumors look like on an MRI?

Choroid plexus tumors usually appear in the ventricles of the brain. They usually have irregular borders and a “cauliflower-like” appearance. The tumors usually enhance with contrast and sometimes many tumors can be seen in a different region of the CNS. Choroid plexus carcinomas may also have some swelling around them, which can be seen during an MRI. Hydrocephalus is almost always seen, too.

What causes choroid plexus tumors?

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 choroid plexus tumors is not known. Genetic changes have been linked to the formation of some choroid plexus tumors. Rarely, certain gene changes that can be passed down through families have been linked to a higher chance of developing choroid plexus carcinomas.

Where do choroid plexus tumors form?

Choroid plexus tumors arise from a structure in the brain called the choroid plexus. It lines the ventricles (fluid-filled cavities) of the brain and its primary function is to produce cerebrospinal fluid (CSF). Choroid plexus tumors almost always form within the ventricles. They can also form in other regions of the CNS.

Do choroid plexus tumors spread?

Choroid plexus tumors can spread to other areas of the CNS through CSF.

What are the symptoms of a choroid plexus tumor?

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

Choroid Plexus Tumors Symptoms

People with choroid plexus tumors may have increased pressure within the skull due to the 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

Who is diagnosed with choroid plexus tumors?

Choroid plexus tumors occur in both children and adults, but are more common in children in the first year of life. Choroid plexus tumors occur slightly more often in females than males. An estimated 1,423 people are living with this tumor in the United States.

What is the prognosis of choroid plexus tumors?

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

Choroid Plexus Tumors Prognosis

The relative 5-year survival rate for choroid plexus tumors is 63.9% 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 choroid plexus tumors?

The first treatment for choroid plexus tumors 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.

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.

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.

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.

A meningioma is a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. Overall, meningiomas are the most common type of primary brain tumor. However, higher grade meningiomas are very rare.

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 meningiomas?

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.

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

1. Grade 1 meningiomas are low grade tumors and are the most common. This means the tumor cells grow slowly.
2. Grade 2 atypical meningiomas are mid-grade tumors. This means the tumors have a higher chance of coming back after being removed. The subtypes include choroid and clear cell meningioma.
3. Grade 3 anaplastic meningiomas are malignant (cancerous). This means they are fast-growing tumors. The subtypes include papillary and rhabdoid meningioma.

What do atypical and anaplastic meningiomas look like on an MRI?

Grade II and III meningiomas usually appear as an enhancing mass on the outside lining of the brain tissue, which may or may not brighten with contrast. Malignant meningiomas can also invade into the brain tissue.

What causes atypical and anaplastic meningiomas?

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 meningiomas is not known. Exposure to radiation, especially in childhood, is the only known environmental risk factor for developing meningiomas. People who have a genetic condition, called neurofibromatosis type 2, are at increased risk for developing meningiomas.

Where do atypical and anaplastic meningiomas form?

Meningiomas form along the dura mater, the outermost layer of tissue that covers and protects the brain and spinal cord. The dura mater is one of three layers that form the meninges. Meningiomas arise from meningeal cells. As a result, they tend to occur along the surface of the brain.

Do atypical and anaplastic meningiomas spread?

Meningiomas can spread to other areas of the CNS through cerebrospinal fluid (CSF). Grade II meningiomas can invade surrounding tissue, including nearby bone tissue. Grade III meningiomas have irregular cells and are likely to invade the brain or spread to other organs in the body.

What are the symptoms of atypical and anaplastic meningiomas?

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

Meningioma Symptoms

• Vision changes
• Loss of hearing or smell
• Confusion
• Seizures
• Headaches that are worse in the morning

Who is diagnosed with atypical and anaplastic meningiomas?

Meningiomas are more common in females, but grades II and III occur more often in males. They are most common in black people, followed by white people, and then Asian-Pacific Islanders. The meningiomas tend to occur in people around 60 years old, with the risk increasing with age. An estimated 2,692 people are living with this tumor in the United States.

What is the prognosis of atypical and anaplastic meningiomas?

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

Atypical and Anaplastic Meningioma Prognosis

The relative 5-year survival rate for atypical and anaplastic meningioma is 63.8% 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 the treatment options for atypical and anaplastic meningiomas?

The first treatment for a malignant meningioma 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 atypical and anaplastic meningiomas receive further treatments. After surgery, radiation is often recommended to delay the return of grade II and III meningiomas. Treatments may also include 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. The role of chemotherapy or clinical trials after radiation therapy is unclear. 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.

Pineal region tumors are primary central nervous system (CNS) tumors. These tumors begin in the brain (in the pineal gland) but can spread to the 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 pineal region tumors?

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.

Pineal region tumors are grouped in four grades and subtypes based on their characteristics (grade 1, 2, 3, or 4 also written as grade I, II, III, IV).

1. Grade 1 pineocytoma are low grade tumors. This means the tumor cells grow slowly.
2. Grade 2 or 3 pineal parenchymal or papillary tumors of the pineal region are both mid-grade tumors. This means the tumors have a higher chance of coming back after being removed.
3. Grade 4 pineoblastoma are malignant (cancerous). This means they are fast-growing tumors that tend to invade nearby tissue.

What do pineal region tumors look like on a CT scan or MRI?

Pineal region tumors usually appear as a solid mass that brightens with contrast. On a CT scan, calcium may be present. Pineoblastomas may extend into surrounding brain structures.

What causes pineal region tumors?

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 pineal region tumors is not known. Pineoblastomas can occur in people with the inherited genetic disorder bilateral retinoblastoma.

Where do pineal region tumors form?

Pineal region tumors form in the pineal region of the brain. This region is located deep in the middle of the brain. Pineal region tumors arise from stem cells near the pineal gland.

Do pineal region tumors spread?

Pineal region tumors can spread to other areas in the CNS through cerebrospinal fluid (CSF).

What are symptoms of a pineal region tumor?

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

Pineal Region Tumor Symptoms

Pineal region tumors may cause increased pressure inside 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:

• Headaches
• Nausea
• Vomiting
• Difficulty with eye movements
• Difficulty with balance
• Difficulty walking

Who is diagnosed with pineal region tumors?

Pineal region tumors occur in children and young to middle age adults. Pineoblastomas are more common during the first 20 years of life. All tumors, except papillary tumors of the pineal region, occur slightly more often in females than males. They are most common in black people. An estimated 1,297 people are living with this tumor in the United States.

What is the prognosis of pineal region tumors?

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

Pineal Region Tumors Prognosis

The relative 5-year survival rate for pineal region tumors is 69.5% 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 pineal region tumors?

The first treatment for pineal region tumors 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.

Pleomorphic xanthoastrocytoma (PXA) is a very rare type of astrocytoma, a primary central nervous system (CNS) tumor. This means it begins in the brain or spinal cord. PXAs typically have genetic alterations, such as the BRAFV600E mutation, that affect a series of chemical reactions known as the MAPK signaling pathway. These genetic alterations can ultimately help the tumor cells grow and survive. While these genetic changes help form and maintain the tumor, they may also be targeted for 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 PXA?

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.

These tumors 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 PXAs are low grade tumors. This means the tumor cells grow slowly.
2. Grade 3 APXAs are malignant (cancerous). This means they are fast-growing tumors.

What do PXAs look like on an MRI?

PXAs often form fluid-filled cysts in a mural nodule, which means a cyst within solid tissue with a dense appearance on MRI. Grade III PXAs may appear similar to PXAs, but the way they look varies and makes it difficult to tell apart from other primary CNS tumors. The diagnosis of PXA can’t be made by imaging alone because they may look the same as glioblastomas and other high-grade brain cancers.

What causes PXAs?

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 PXAs is not known. People with gene changes that can be passed down through families such as neurofibromatosis type I and familial melanoma/astrocytoma syndrome, are at increased risk for developing PXAs.

Where do PXAs form?

PXAs usually occur in one of the four lobes of the brain. The temporal lobe is the most common location. Rarely, PXAs can form in the cerebellum, brainstem, and spinal cord. PXAs are usually located close to the surface of the brain tissue. Scientists believe these tumors arise from cells that grow into glial and nerve cells. PXAs can look a variety of ways under the microscope and can be challenging to diagnose, as they share characteristics with other primary brain tumors. Review by an expert neuropathologist is recommended to confirm this diagnosis. Molecular testing will often reveal a BRAF gene change in these tumors.

Do PXAs spread?

Grade II PXAs are slow-growing tumors that usually don’t spread. Grade III PXAs are fast-growing tumors that usually invade brain tissue in the lobe of the brain where they started. It’s rare for PXAs of any grade to spread to other areas of the CNS. There are no reported cases of spread outside of the CNS.

What are symptoms of a PXA?

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

PXA Symptoms

People with Grade II PXAs may have:

• Seizures
• Epilepsy
• Chronic headaches

People with Grade III PXAs may have:

• Seizures
• Weakness
• Numbness
• Speech and language changes

Who is diagnosed with PXAs?

PXAs can occur at any age, but tend to happen in young adulthood, and occur slightly more often in males than females. PXAs are more common in people of African descent. An estimated 1,081 people are living with this tumor in the United States.

What is the prognosis of PXAs?

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

The relative 5-year survival rate for PXA is 76.2% 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 PXAs?

The first treatment for a PXA 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. Grade II PXAs can often be treated by surgery alone.

If they come back after surgery, there is no standard treatment for PXAs. Possible treatments at this time may include radiation, chemotherapy, targeted therapies, or clinical trials. If a BRAF change is present, clinical trials using a drug to target that may also be an 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.

PNETs are primary central nervous system (CNS) tumors. This means they begin in the brain or spinal cord. PNET is a term that stands for a group of tumors, and these tumors are currently being reclassified and given other specific names based on their molecular features.

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 PNETs?

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.

The group of tumors, formerly known as PNETs, are grade 4 (also written as grade IV) tumors. This means they are malignant (cancerous) and fast-growing. These are tumor types that belong to this group:

• Medulloepithelioma
• CNS neuroblastoma
• CNS ganglioneuroblastoma
• Embryonal tumor with multilayered rosettes and other unspecified embryonal tumors

What do PNETs look like on an MRI?

PNETs usually initially appear as a single mass in the cortex, the outer layer of the brain. PNETs often enhance with contrast and more than one tumor can be identified. Sometimes, there are cysts or fluid collections found within the mass. PNETs may also have some swelling around them.

What causes PNETs?

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 PNETs is not known. Some PNETs are related to genetic changes.

Where do PNETs form?

PNETs are commonly found in the brain and rarely, in the brainstem or spinal cord. PNETs is a group of tumors that form from the ectoderm, the outermost layer of cells of an embryo in early development.

Do PNETs spread?

PNETs can spread to other areas of the CNS and organs. When first diagnosed, about one-third of patients’ tumors have spread.

What are the possible symptoms of PNETs?

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

PNET Symptoms

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

People with PNETs in the spine may have:

• Bowel or bladder incontinence
• Pain in the back and legs

Who is diagnosed with PNETs?

PNETs occur more commonly in children, but can occur in adults. PNETs are very rare tumors, and their classification is evolving. PNETs occur more often in males than females. They are most common in white people. An estimated 950 people are living with this tumor in the United States.

What is the prognosis of PNETs?

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

PNET Prognosis

The relative 5-year survival rate for PNETs cannot be calculated due to small numbers. 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 PNETs?

The first treatment for PNETs 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.

People with PNETs usually receive further treatments. Treatments may include radiation, chemotherapy, or clinical trials. Surgery is usually followed by radiation in patients 3 years old or older, sometimes including the brain and the spine. 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.