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Brain tumor

Brain tumor

A brain tumor is a lump of abnormal cells growing in your brain. When cells grow abnormally they may form a lump called a tumor. Normally, cells multiply and die in an orderly way, so that each new cell replaces one lost. A tumor is a mass of tissue made up of cells that grow and multiply in an abnormal and uncontrolled way. Brain tumors can be benign (not cancer) or malignant (cancer); both cause symptoms that require treatment. Benign tumors are not cancerous. A benign brain tumor grows slowly and stays in one place. It is unlikely to spread to another part of your body. But a benign brain tumor may cause damage just by being there and pressing on your brain or nearby structures. This can be life-threatening, or affect other parts of your body, and may need urgent treatment. A malignant brain tumor is cancerous and it can also be called brain cancer. A malignant brain tumor can spread to other areas of your brain, or your body.

  1. Benign brain tumors are not cancerous. Benign brain and spinal cord tumors grow more slowly and stays in one place, but can press on nearby areas of the brain. A benign brain tumor may cause damage just by being there and pressing on your brain or nearby structures. This can be life-threatening, or affect other parts of your body, and may need urgent treatment. Benign brain and spinal cord tumors rarely spread into other tissues, and may recur (come back) after treatment. Benign brain tumors are low grade (grade 1 or 2), which means they grow slowly and are less likely to return after treatment
  2. Malignant brain tumors are cancerous and are also called brain cancer. Malignant brain tumors are high grade (grade 3 or 4) brain tumors. Malignant brain tumors are considered to be life threatening because they grow rapidly and invade surrounding brain tissue. Malignant brain and spinal cord tumors are likely to grow quickly and spread into other brain tissue or spread to other areas of your body.

There are over 130 types of brain tumors 1. Together with tumors of the spinal cord, they are collectively called central nervous system (CNS) tumors.

  • Some brain tumors are primary brain tumors, which start in your brain.
  • Secondary brain tumors are tumors found in the brain that have started somewhere else in your body. These are also known as metastatic brain tumors or brain metastases. Metastatic brain tumors are more common than primary brain tumors.

Your brain controls all the parts of your body and its functions and produces your thoughts. Depending on where it is, a tumor in your brain can affect these functions. Slow growing brain tumors may not have any symptoms to start with. Some symptoms may also be caused by the treatments used to manage the tumor.

Many brain tumor symptoms and brain cancer symptoms are similar to those of other diseases and conditions.

Brain tumors can cause many symptoms, depending on where the tumor is, how big it is and what type of tumor it is. Some of the most common are:

  • Headaches, often in the morning
  • Nausea and vomiting
  • Changes in your ability to talk, hear, or see
  • Problems with balance or walking
  • Problems with thinking or memory
  • Feeling weak or sleepy
  • Changes in your mood or behavior
  • Seizures

The American Cancer Society’s estimates for brain and spinal cord tumors in the United States for 2022 include both adults and children 2, 3:

  • New cases: About 25,050 malignant tumors of the brain or spinal cord (14,170 in males and 10,880 in females) will be diagnosed. These numbers would be much higher if benign (non-cancer) tumors were also included.
  • Deaths: About 18,280 people (10,710 males and 7,570 females) will die from brain and spinal cord tumors.
  • 5-Year Relative Survival: 32.5%. Relative survival is an estimate of the percentage of patients who would be expected to survive the effects of their brain and spinal cord tumors. It excludes the risk of dying from other causes. Because survival statistics are based on large groups of people, they cannot be used to predict exactly what will happen to an individual patient. No two patients are entirely alike, and treatment and responses to treatment can vary greatly.
  • Brain and spinal cord tumors deaths as a percentage of All Cancer Deaths: 3%.
  • Rate of New Cases and Deaths per 100,000: The rate of new cases of brain and other nervous system cancer was 6.3 per 100,000 men and women per year. The death rate was 4.4 per 100,000 men and women per year. These rates are age-adjusted and based on 2015–2019 cases and deaths.
  • Lifetime Risk of Developing Cancer: Approximately 0.6 percent of men and women will be diagnosed with brain and other nervous system cancer at some point during their lifetime, based on 2017–2019 data.
  • In 2019, there were an estimated 176,566 people living with brain and other nervous system cancer in the United States.

Data from the Surveillance, Epidemiology, and End Results (SEER) Program database for 2013 to 2017 indicated that the combined incidence of brain and other central nervous system (CNS) tumors in the United States was 6.4 per 100,000 persons per year. Data for 2014 to 2018 indicated an estimated mortality rate of 4.4 deaths per 100,000 persons per year  3. Worldwide, approximately 308,102 new cases of brain and other central nervous system (CNS) tumors were diagnosed in the year 2020, with an estimated 251,329 deaths 4.

Overall, the chance that a person will develop a malignant tumor of the brain or spinal cord in their lifetime is less than 1%. In general, the incidence of primary central nervous system (CNS) tumors is higher in White individuals than in Black individuals 3. The risk of developing any type of brain or spinal cord tumor is slightly higher among women than among men, although the risk of developing a malignant tumor is slightly higher for men than for women and the mortality is higher in males than in females 3. This is largely because certain types of tumors are more common in one gender or the other (for example, meningiomas are more common in women).

Doctors diagnose brain tumors by doing a neurologic exam and tests including an MRI, CT scan, and biopsy.

When diagnosed with a brain tumor, it is important to understand:

  1. The type and grade of the brain tumor (how aggressive it is)
  2. Whether it is a primary or a secondary brain tumor
  3. If it is cancerous (malignant) or benign brain tumor
  4. Where in the brain the tumor is located

Brain tumor treatment options include watchful waiting, surgery, radiation therapy, chemotherapy, and targeted therapy. Targeted therapy uses drugs or other substances that attack cancer cells with less harm to normal cells. Many people get a combination of treatments.

Survival rates for brain and spinal cord tumors vary widely, depending on the type of tumor (and other factors).

Human brain

To understand brain and spinal cord tumors, it helps to know about the normal structure and function of the central nervous system (CNS), which is the medical name for the brain and spinal cord. The human brain is a component of the central nervous system (CNS). The human brain is roughly the size of two clenched fists and weighs about 1.6 kg (3.5 lb) in men and 1.45 kg in women 5. The difference between the sexes is proportional to body size, not intelligence.

The brain is the center of thought, feeling, memory, speech, vision, hearing, movement, breathing, and much more. The spinal cord and special nerves in the head called cranial nerves help carry messages between the brain and the rest of the body. These messages tell our muscles how to move, transmit information gathered by our senses, and help coordinate the functions of our internal organs.

The brain is protected by the skull. Likewise, the spinal cord is protected by the bones (vertebrae) of the spinal column.

The brain and spinal cord are surrounded and cushioned by a special liquid, called cerebrospinal fluid (CSF). Cerebrospinal fluid is made by the choroid plexus, which is found in spaces within the brain called ventricles. The ventricles and the spaces around the brain and spinal cord are filled with CSF.

Anatomists conceptually divide the brain into four major parts:

  • The Cerebrum. The cerebrum controls thinking, learning, problem solving, emotions, speech, reading, writing, and voluntary movement.
  • The Diencephalon (thalamus, hypothalamus, and epithalamus).
  • The Cerebellum. The cerebellum controls movement, balance, and posture.
  • The Brainstem. The brain stem connects the brain to the spinal cord, and controls breathing, heart rate, and the nerves and muscles that we use to see, hear, walk, talk, and eat.

Together, the brain and spinal cord (the central nervous system [CNS]) control the physiological and psychological functions of your body.

The brain, like the spinal cord, is composed of gray and white matter. Gray matter—the seat of the neurosomas, dendrites, and synapses—forms a surface layer called the cortex over the cerebrum and cerebellum, and deeper masses called nuclei surrounded by white matter. White matter lies deep to the cortical gray matter in most of the brain, opposite from the relationship of gray and white matter in the spinal cord. As in the spinal cord, white matter is composed of tracts, or bundles of axons, which here connect one part of the brain to another and to the spinal cord.

Figure 1. Human brain

Human brain

Figure 2. Medial aspect of the human brain

human brain anatomy

Figure 3. Brain structures and brain functions

Brain structures and brain functions

Figure 4. Ventricles of the brain

Ventricles of the brain

Figure 5. Cerebrospinal fluid (CSF) formation, absorption and circulation around and within the brain

CSF formation, absorption and circulation around and within the brain

Cerebrum

The cerebrum is the large, outer part of the brain. The cerebrum is about 83% of the brain’s volume and consists of a pair of half globes called the cerebral hemispheres (left and right hemispheres). Each hemisphere is marked by thick folds called gyri (singular, gyrus) separated by shallow grooves called sulci (singular, sulcus). A very deep median groove, the longitudinal fissure, separates the right and left hemispheres from each other. At the bottom of this fissure, the hemispheres are connected by a thick bundle of nerve fibers called the corpus callosum—a prominent landmark for anatomical description with a distinctive C shape in sagittal section.

The cerebral hemispheres control reasoning, thought, emotion, and language. It is also responsible for planned (voluntary) muscle movements (throwing a ball, walking, chewing, etc.) and for taking in and interpreting sensory information such as vision, hearing, smell, touch, and pain.

Cerebellum

The cerebellum occupies the posterior cranial fossa inferior to the cerebrum, separated from it by the transverse cerebral fissure. It is also marked by fissures, sulci, and gyri (called folia in the cerebellum). The cerebellum is the second-largest region of the brain, constituting about 10% of its volume but containing over 50% of its neurons.

The cerebellum lies under the cerebrum at the back part of the brain. It helps coordinate movement. Tumors of the cerebellum can cause problems with coordination in walking, trouble with precise movements of hands, arms, feet, and legs, problems swallowing or synchronizing eye movements, and changes in speech rhythm.

Brain stem

The brain stem is the lower part of the brain that connects to the spinal cord. It contains bundles of very long nerve fibers that carry signals controlling muscles and sensation or feeling between the cerebrum and the rest the body. Special centers in the brain stem also help control breathing and the beating of the heart. Also, most cranial nerves (see Figures 1 and 2) start in the brain stem.

The brainstem is defined all of the brain except the cerebrum and cerebellum. The brain stem is divided into 3 main parts, from rostral (front towards the nose) to caudal (back end), are the midbrain, pons, and medulla oblongata. In a living person, the brainstem is oriented like a vertical stalk with the cerebrum perched on top like a mushroom cap. Postmortem changes give it a more oblique angle in the cadaver and consequently, in many medical illustrations. Towards the back end, the brainstem ends at the foramen magnum of the skull, and the central nervous system (CNS) continues below this as the spinal cord.

Tumors in this critical area of the brain can cause weakness, stiff muscles, or problems with sensation, facial or eye movement, hearing, or swallowing. Double vision is a common early symptom of brain stem tumors, as are problems with coordination in walking. Because the brain stem is a small area that is so essential for life, it might not be possible to surgically remove tumors in this area.

Cranial nerves

The cranial nerves extend directly out of the base of the brain (as opposed to coming out of the spinal cord). These nerves carry signals directly between the brain and the face, eyes, ears, tongue, mouth, and some other areas.

Tumors starting in cranial nerves can cause vision problems, trouble swallowing, hearing loss in one or both ears, or facial paralysis, numbness, or pain.

Spinal cord

The spinal cord has bundles of very long nerve fibers that carry signals that control muscles, sensation or feeling, and bladder and bowel control.

Spinal cord tumors can cause weakness, paralysis, or numbness. The spinal cord is a narrow structure, so tumors within it usually cause symptoms on both sides of the body (for example, weakness or numbness of both legs). This is different from most brain tumors, which often affect only one side of the body.

The nerves that reach the arms begin in the spinal cord at the level of the neck (cervical spine). Nerves that branch off the spinal cord to the legs, bowel, and bladder arise in the back (thoracic and lumbar spine). Most tumors of the spinal cord start in the neck and can cause symptoms in the arms and legs, as well as affect bowel and bladder function. Spinal cord tumors below the neck only affect the legs and bowel and bladder function.

Pituitary gland and hypothalamus

The pituitary is a small gland at the base of the brain. It is connected to a part of the brain called the hypothalamus. They work together to make hormones that help regulate the activity of several other glands in the body. For example:

  • They control the amount of thyroid hormone made by the thyroid gland.
  • They affect breast milk production and release.
  • They control the amount of male or female hormones made by the testicles or ovaries.
  • They make growth hormone, which stimulates body growth.
  • They make vasopressin, which regulates water balance by the kidneys.

The growth of tumors in or near the pituitary or hypothalamus, as well as surgery and/or radiation therapy in this area, can affect these functions. For example, tumors starting in the pituitary gland sometimes make too much of a certain hormone, which can cause problems. On the other hand, a person may have low levels of one or more hormones after treatment and may need to take hormones to make up for this.

Blood-brain barrier

The inner lining of the small blood vessels (capillaries) in the brain and spinal cord creates a very selective barrier between the blood and the tissues of the central nervous system. This barrier normally helps maintain the brain’s metabolic balance and keeps harmful toxins from getting into the brain. Unfortunately, it also keeps out most chemotherapy drugs that are used to kill cancer cells, which in some cases limits their usefulness.

Choroid plexus

The choroid plexus is the area of the brain within the ventricles that makes CSF, which nourishes and protects the brain.

Pineal gland

The pineal gland is not really part of the brain. The pineal gland is a small endocrine gland that sits between the cerebral hemispheres. It makes melatonin, a hormone that regulates sleep, in response to changes in light. The most common tumors of the pineal gland are called pineoblastomas.

Types of brain tumors

Doctors refer to a brain tumor based on where the tumor cells originated and whether they are cancerous (malignant) or not (benign). There are over 130 types of brain and central nervous system (CNS) tumors. Brain and spinal cord tumors are different for everyone. They form in different areas, develop from different cell types, and may have different treatment options. However, there are two main types of brain and spinal cord tumors:

  1. Primary brain (or spinal cord) tumors are tumors that start in your brain or spinal cord. Primary brain (or spinal cord) tumors develop inside the brain or spinal cord, and almost never spread outside of the brain or spine. Primary brain tumors are most commonly named after the cells from which they derive. Unlike cancers that start in other parts of your body, tumors that start in the brain or spinal cord rarely spread to distant organs. Even so, brain or spinal cord tumors are rarely considered benign (non-cancerous). They can still cause damage by growing and spreading into nearby areas, where they can destroy normal brain tissue. And unless they are completely removed or destroyed, most brain or spinal cord tumors will continue to grow and eventually be life-threatening.
    • Some common types of primary brain tumors are:
      1. Gliomas (including astrocytomas and glioblastomas) which start in glial cells
      2. Medulloblastomas — which start in the cerebellum
      3. Meningiomas — which start in the meninges
      4. CNS (central nervous system) Lymphoma
  2. Secondary brain (or spinal cord) tumors are tumors found in the brain that have started somewhere else in your body. These are also known as metastatic brain tumors or brain metastases. In adults, metastatic brain tumors are actually more common than primary brain tumors, and they are treated differently. The types of cancer that most commonly spread to the brain include melanoma, bowel, breast, kidney and lung cancers.

Brain tumors are also divided into benign (not cancer) brain tumor or malignant (cancer) brain tumor.

  1. Benign brain tumors are not cancerous. Benign brain and spinal cord tumors grow more slowly and stays in one place, but can press on nearby areas of the brain. A benign brain tumor may cause damage just by being there and pressing on your brain or nearby structures. This can be life-threatening, or affect other parts of your body, and may need urgent treatment. Benign brain and spinal cord tumors rarely spread into other tissues, and may recur (come back) after treatment. Benign brain tumors are low grade (grade 1 or 2), which means they grow slowly and are less likely to return after treatment
  2. Malignant brain tumors are cancerous and are also called brain cancer. Malignant brain tumors are high grade (grade 3 or 4) brain tumors. Malignant brain tumors are considered to be life threatening because they grow rapidly and invade surrounding brain tissue. Malignant brain and spinal cord tumors are likely to grow quickly and spread into other brain tissue or spread to other areas of your body.

Today, most medical institutions use the World Health Organization (WHO) classification system to identify brain tumors 1. The WHO classifies brain tumors by cell origin and how the cells behave, from the least aggressive (benign) to the most aggressive (malignant). Some tumor types are assigned a grade, ranging from Grade 1 (least malignant) to Grade 4 (most malignant), which signifies the rate of growth. There are variations in grading systems, depending on the tumor type. The classification and grade of an individual tumor help predict its likely behavior. This section describes the most frequently diagnosed types.

World Health Organization Brain Tumor Classification

World Health Organization Brain Tumor Classification
[Source 1 ]

Gliomas

Gliomas are brain tumor that start in glial cells. These are the supporting cells of the brain and the spinal cord. There are different types of gliomas.

There are 3 types of glial cells:

  1. Astrocytes – tumor that start in these cells are called astrocytoma or glioblastoma
  2. Oligodendrocytes – tumor that start in these cells are called oligodendrogliomas
  3. Ependymal cells – tumor that start in these cells are called ependymomas

About 3 out of 10 of all brain tumors are gliomas. Most fast-growing brain tumors are gliomas. The most common type is called astrocytoma.

Astrocytomas

Astrocytomas are tumors that start in glial cells called astrocytes. Astrocytes are star shaped cells. They support the nerve cells (neurones) in the brain. Astrocytomas are the most common type of brain tumors in both adults and children. About 2 out of 10 brain tumors are astrocytomas. Symptoms of astrocytoma depend on where the tumor is in the brain. Common symptoms include headaches and seizures (fits).

Most astrocytomas can spread widely throughout the brain and blend with the normal brain tissue, which can make them very hard to remove with surgery. Sometimes they spread along the cerebrospinal fluid (CSF) pathways. It is very rare for them to spread outside of the brain or spinal cord.

As with other types of brain tumors, astrocytomas are often grouped by grade (according to how quickly they are likely to grow).

  • Low-grade (grade 1 or 2) astrocytomas tend to grow slowly. These include:
    • Non-infiltrating (grade 1) astrocytomas, which do not usually grow into nearby tissues and tend to have a good prognosis. Examples include pilocytic astrocytomas and subependymal giant cell astrocytomas (SEGAs). These are more common in children than in adults.
    • Grade 2 astrocytomas, such as diffuse astrocytomas and pleomorphic xanthoastrocytomas (PXAs). These tumors tend to be slow growing, but they can grow into nearby areas, which can make them harder to remove with surgery. These tumors can become more aggressive and faster growing over time.
  • High-grade (grade 3 or 4) astrocytomas tend to grow quickly and spread into the surrounding normal brain tissue. These include:
    • Anaplastic (grade 3) astrocytomas
    • Glioblastomas (grade 4) are also called glioblastoma multiforme (GBM), which are the fastest growing. These tumors make up more than half of all gliomas and are the most common malignant brain tumors in adults.

Oligodendrogliomas

These tumors start in brain glial cells called oligodendrocytes. Oligodendroglioma is a rare type of brain tumor. Only about 1% to 2% of brain tumors are oligodendrogliomas. Oligodendrogliomas are more common in adults but can also start in young children. Symptoms of oligodendroglioma depend on where the tumor is in the brain. Common symptoms include headaches and seizures (fits).

Oligodendrogliomas are grade 2 tumors that tend to grow slowly, but most of them can grow into (infiltrate) nearby brain tissue and can’t be removed completely by surgery. Oligodendrogliomas sometimes spread along the cerebrospinal fluid (CSF) pathways but rarely spread outside the brain or spinal cord. As with astrocytomas, they can become more aggressive over time. Very aggressive (grade 3) forms of these tumors are known as anaplastic oligodendrogliomas.

Treatment depends on whether the oligodendroglioma is low grade (slow growing) or high grade (fast growing). It also depends on whether you have changes (mutations) in certain genes.

  • Treatment for low grade oligodendroglioma
    • Surgery. Surgery is usually the first treatment you have. The exact type of surgery you have depends on where the tumor is. A highly specialist doctor (neurosurgeon) removes as much of the tumor as possible. Oligodendrogliomas tend to grow into the brain tissue surrounding the tumor. This means that they don’t have a clear border between the tumor and the normal brain tissue. Not having a clear border can make it difficult to remove the tumor completely.
    • Active monitoring. Your doctor might monitor you with regular MRI scans. This is called active monitoring. You may have active monitoring if you have a very slow growing oligodendroglioma.
    • Radiation therapy. You might have radiotherapy after surgery. Radiotherapy uses high energy x-rays to destroy cancer cells. You may have radiotherapy after surgery if:
      • you have a gene change called a 1p/19q co-deletion
      • you are over 40 years old
      • doctors can see tumor left behind after surgery
    • Chemotherapy. You might have chemotherapy before or after radiotherapy. Chemotherapy uses cytotoxic drugs to destroy cancer cells. You usually have a combination of chemotherapy drugs called PCV. It includes the drugs:
      • procarbazine
      • lomustine (CCNU)
      • vincristine
  • Treatment for high grade oligodendroglioma
    • Surgery. Surgery is the main treatment for high grade oligodendroglioma. A specialist doctor called neurosurgeon removes as much of the tumor as possible. This is called debulking. It is not usually possible to remove all of the tumor. Your doctor might recommend you have radiotherapy and chemotherapy afterwards.
    • Radiation therapy. Radiotherapy uses high energy x-rays to destroy cancer cells. You might have radiotherapy after surgery if you are well overall and able to care for yourself.
    • Chemotherapy uses cytotoxic drugs to destroy cancer cells. You might have a combination of chemotherapy drugs called PCV before or after radiotherapy. PCV includes the
      • drugs:procarbazine
      • lomustine (CCNU)
      • vincristine

Ependymomas

Ependymomas start in ependymal cells, and typically grow in the ventricles or spinal cord in adults. Ependymomas can range from fairly low-grade (grade 2) tumors to higher grade (grade 3) tumors, which are called anaplastic ependymomas. Ependymomas are rare tumors. Only about 2% of brain tumors are ependymomas.

Ependymomas are more common in children and young adults but can also happen in older people. In older people, ependymomas tend to start in the lower part of the spinal cord.

Ependymomas are more likely to spread along the cerebrospinal fluid (CSF) pathways than other gliomas but do not spread outside the brain or spinal cord. Ependymomas may block the flow of CSF from the ventricles, causing the ventricles to become very large – a condition called hydrocephalus.

Unlike astrocytomas and oligodendrogliomas, ependymomas usually do not grow into normal brain tissue. As a result, some (but not all) ependymomas can be removed completely and cured by surgery. But because they can spread along ependymal surfaces and CSF pathways, treating them can sometimes be difficult. Spinal cord ependymomas have the greatest chance of being cured with surgery, but treatment can cause side effects related to nerve damage.

Brain stem glioma

Glioma in the brain stem is very rare. The brain stem is the lowest part of the brain, that connects with the spinal cord. It controls body functions that you don’t usually think about such as your breathing.

Brain stem gliomas are more common in children than in adults. For some children, they grow rapidly and spread to other parts of the brain. Brain stem gliomas in children are also called diffuse intrinsic pontine glioma.

For a brain stem glioma, you usually have surgery to remove part of the tumor. After surgery, you might have radiotherapy and chemotherapy, or chemotherapy on its own.

Meningiomas

Meningiomas begin in the meninges, the layers of tissue that surround the outer part of the brain and spinal cord. The meninges are membranes that support and protect the brain and spinal cord. A clear fluid called cerebrospinal fluid (CSF) travels in the spaces formed by the meninges. Meningiomas account for about 1 out of 3 primary brain and spinal cord tumors. They are the most common primary brain tumors in adults (although strictly speaking, they are not actually brain tumors). Meningiomas can start anywhere in the brain and spinal cord. But they are more common in the cerebrum and cerebellum.

The risk of these tumors increases with age. They occur about twice as often in women. Sometimes these tumors run in families, especially in those with neurofibromatosis, a syndrome in which people develop many benign tumors of nerve tissue.

Meningiomas are often assigned a grade, based on how the cells look under the microscope:

  • Grade 1 (benign) meningiomas have cells that look the most like normal cells. These are the most common type of meningioma. Most of these tumors can be cured by surgery, but some grow very close to vital structures in the brain or cranial nerves and cannot be cured by surgery alone.
  • Grade 2 (atypical or invasive) meningiomas usually have cells that look slightly more abnormal. They can grow directly into nearby brain tissue and bone and are more likely to come back (recur) after surgery.
  • Grade 3 (anaplastic or malignant) meningiomas have cells that look the most abnormal. These are the least common type of meningiomas. They tend to grow quickly, can grow into nearby brain tissue and bone, and are the most likely to come back after treatment. Some may even spread to other parts of the body.

Symptoms of meningioma depend on where the tumor is in the brain. Symptoms include:

  • seizures (fits)
  • weakness
  • loss of eyesight
  • hearing loss

Meningiomas treatment depends on whether the meningioma is low grade (slow growing) or high grade (fast growing). It also depends on where the tumor is.

  • Active monitoring. For a low grade meningioma, your doctor might monitor you with regular MRI scans. This is called active monitoring. You then have treatment if there are signs that the tumor is growing.
  • Surgery. A highly specialized doctor (neurosurgeon) removes as much of the tumor as possible. Sometimes this is the only treatment you need. The exact type of surgery you have depends on where the tumor is. It isn’t always possible to completely remove the tumor during the operation. Especially if the tumor is growing around important nerves or blood vessels. You might have more surgery if doctors couldn’t remove all of the tumor. Or your doctor might suggest that you have radiotherapy.
  • Radiation therapy. Radiotherapy uses high energy x-rays to kill tumor cells. You might have radiotherapy:
    • to reduce the risk of the meningioma coming back, especially if the tumor is fast growing
    • if the tumor is in an area that is too difficult to operate (for example, the base of the skull)
    • if you can’t have surgery for any reason
    • if the meningioma comes back
    • You may have a type of radiotherapy called stereotactic radiotherapy. It targets high doses of radiotherapy to the tumor.

Medulloblastomas

Medulloblastomas develop from neuroectodermal cells (early forms of nerve cells) in the cerebellum. Medulloblastomas are part of a class of tumors called embryonal tumors that can also start in other parts of the central nervous system. Medulloblastomas are fast-growing (grade 4) tumors and often spread throughout the CSF pathways. Between 33 to 35 out of 100 children (33 – 35%) have medulloblastoma that has spread when they are first diagnosed, but they can be treated by surgery, radiation therapy, and chemotherapy.

Medulloblastomas occur much more often in children than in adults. Medulloblastoma is the second most common brain tumor in children. But it is the most common malignant (high grade) children’s brain tumor.

Medulloblastoma is most commonly diagnosed in children between 3 and 4 and between 8 and 10 years old. And it’s slightly more common in boys than girls.

Around 5 out of every 100 cases of medulloblastoma (around 5%) are linked with either:

  • Familial Adenomatous Polyposis (FAP) – sometimes known as Turcot syndrome
  • Nevoid basal cell carcinoma syndrome (NBCCS) – also known as Gorlin syndrome

Researchers are learning more about medulloblastoma all the time. At the moment, medulloblastoma is grouped by what the cells look like under the microscope. Your child’s doctors need a sample of the tumor to work out which type it is. This means they won’t be able to tell you straight away about what type or group it is.

There are 4 groups of medulloblastoma:

  1. Classical medulloblastoma – around 8 out of 10 children (around 80%) have classical medulloblastoma
  2. Anaplastic or large cell medulloblastoma
  3. Nodular or desmoplastic medulloblastoma – this type is most common in infants
  4. Medulloblastoma with extensive nodularity (MBEN)

Using new techniques, scientists can also look at the genes and proteins inside the medulloblastoma cells. This means doctors can now group medulloblastoma into 4 further subtypes. These are:

  • Subtype 1 – wingless (WNT) medulloblastoma
  • Subtype 2 – sonic hedgehog (SHH) medulloblastoma
  • Subtype 3 – group 3 medulloblastoma
  • Subtype 4 – group 4 medulloblastoma

Researchers are interested in learning more about these differences in medulloblastoma. They hope to make treatments targeted to these gene changes in the future.

Your child might have symptoms for a few weeks or months before they are diagnosed with medulloblastoma. Many of the symptoms are general and non specific. Some are similar to less serious childhood illnesses. Symptoms include:

  • headaches in the morning
  • feeling or being sick – being sick often makes the headaches feel better
  • double vision
  • finding it hard to sit or stand unsupported – your child might often fall backwards
  • being more fractious or irritable – it might be taking longer than usual to get your young child to settle
  • loss of appetite
  • behavior changes – they might be interacting with you or their siblings less

A symptom that is sometimes found during a young infants routine development check up is an increasing head size (head circumference). Or the soft spot on top of the skull (fontanelle) might be swollen.

The main treatments for medulloblastoma are:

  • surgery
  • radiotherapy
  • chemotherapy

Each child has their own treatment plan. Surgery is usually the first treatment your child will have. Less commonly your child might have chemotherapy first to shrink the tumour before surgery.

Your child’s specialist team decides on the best treatment for your child. Both you and your child will be involved in decisions about treatment and care.

Gangliogliomas

Gangliogliomas contain both neurons and glial cells. These tumors are very uncommon in adults. They are typically slow growing (grade I) tumors and can usually be cured by surgery alone or surgery combined with radiation therapy.

Schwannomas (neurilemmomas)

Schwannomas develop from Schwann cells also called the nerve sheath (the tissue that covers nerves), which surround and insulate cranial nerves and other nerves. Schwannomas make up about 8% of all central nervous system (CNS) tumors.

Schwannomas are almost always benign (grade 1) tumors. They can arise from any cranial nerve. When they form on the cranial nerve responsible for hearing and balance near the cerebellum, they are called vestibular schwannomas or acoustic neuromas. Neurofibromatosis Type 2 (NF2) is a rare inherited condition which can make you more likely to develop vestibular schwannomas in both ears.

Schwannomas can also start on spinal nerves after the point where they have left the spinal cord. When this happens, they can press on the spinal cord, causing weakness, sensory loss, and bowel and bladder problems.

When schwannomas are cancerous they are called malignant schwannomas. Malignant schwannomas are also called malignant peripheral nerve sheath tumors (MPNSTs) or neurofibrosarcomas. Malignant schwannomas can start anywhere in the body. But the most common area is the major nerve of the leg (the sciatic nerve).

Treatment of Schwannomas depends on where the tumor is in your body and whether it is non cancerous (benign) or cancerous (malignant). Your specialist may monitor you carefully at first rather than suggest treatment. They will see you regularly and monitor any symptoms you have. This is because the treatment can cause a lot of side effects.

  • Surgery. The main treatment for schwannoma is surgery to remove the tumor. The type of surgery you have depends on where in the body the tumor is.
  • Targeted radiotherapy. For small vestibular schwannomas, you may have stereotactic radiotherapy or radiosurgery. Both treatments target high doses of radiotherapy to the tumor. You might have targeted radiotherapy alone, or after surgery if the surgeon was unable to completely remove the tumor.
  • Treatment for malignant schwannoma. You might also have radiotherapy and chemotherapy if you have a malignant tumor.

Craniopharyngiomas

Craniopharyngiomas are slow-growing (grade 1) tumors start above the pituitary gland but below the brain itself. Craniopharyngiomas may press on the pituitary gland and the hypothalamus, causing hormone problems. Because they start very close to the optic nerves, they can also cause vision problems. Their tendency to stick to these important structures can make them hard to remove completely without damaging vision or hormone balance. Craniopharyngiomas are more common in children, but they are sometimes seen in adults.

Chordomas

Chordomas are rare tumors start in the bone at the base of the skull or at the lower end of the spine. Chordomas don’t start in the central nervous system, but they can injure the nearby brain or spinal cord by pressing on it.

These tumors are treated with surgery if possible, often followed by radiation therapy, but they tend to come back in the same area after treatment, causing more damage. Chordomas usually do not spread to other organs.

Non-Hodgkin lymphomas

Lymphomas are cancers that start in white blood cells called lymphocytes (one of the main cell types of the immune system). Most lymphomas start in other parts of the body, but some start in the CNS, and are called primary CNS lymphomas.

Non-Hodgkin lymphomas (NHL) are more common in people with immune system problems, such as those infected with HIV, the virus that causes AIDS. Because of new treatments for AIDS, primary CNS lymphomas have become less common in recent years. They account for about 2% of primary brain tumors.

Non-Hodgkin lymphomas (NHL) often grow quickly and can be hard to treat. Recent advances in chemotherapy, however, have improved the outlook for people with these cancers.

Pituitary tumors

Pituitary tumors are tumors that start in the pituitary gland and they are almost always benign (non-cancerous). But they can still cause problems if they grow large enough to press on nearby structures or if they make too much of any kind of hormone.

Hemangioblastomas

Hemangioblastomas are rare benign brain tumors that start in the cells that line the blood vessels in the brain, spinal cord and brain stem. Hemangioblastomas usually grow slowly over some years. Most hemangioblastomas start in the back of the brain (cerebellum).

Hemangioblastomas are rare tumors. About 2 out of every 100 brain tumors (2%) diagnosed are hemangioblastomas.

Hemangioblastomas don’t usually spread to other parts of the brain. The symptoms you have depend on where the tumor is in the brain. Symptoms might include:

  • problems with walking, balance, speech and coordination
  • a build up of brain fluid (cerebrospinal fluid) which can cause headaches and feeling sick

Some hemangioblastomas happen because of a rare inherited syndrome called von Hippel-Lindau syndrome. People with this syndrome have a higher risk of developing different types of tumors, including hemangioblastomas.

Hemangioblastomas treatment depends on:

  • the size of the tumor
  • the symptoms you have
  • your age
  • your quality of life

Treatment might be monitoring, surgery or radiotherapy. The team caring for you will talk to you about your treatment.

  • Active monitoring or watchful waiting. You might not need treatment straight away if you have a very small hemangioblastoma and you don’t have symptoms. If this is the case, your doctors may recommend monitoring the tumor with regular MRI scans.
  • Surgery. Surgery is the main treatment for hemangioblastoma. A brain specialist surgeon (neurosurgeon) removes all the tumor or just a part. This depends on where the tumor is. You might have side effects after the operation. The side effects include:
    • bleeding in the brain
    • infection in the membranes that surround the brain (meningitis)
  • Radiotherapy. You might have radiotherapy if:
    • you can’t have surgery for any reason
    • your hemangioblastoma has come back after surgery
    • doctors couldn’t remove all the tumor during the operation
  • You might have a type of radiotherapy called stereotactic radiotherapy or radiosurgery. Both treatments target high doses of radiotherapy to the tumor.

Brain tumor signs and symptoms

The signs and symptoms of brain tumors differ from person to person. Some people may have symptoms that suggest there is a brain tumor, others have no obvious symptoms. Signs and symptoms depend on where the tumor forms in the brain, what the affected part of the brain controls, and the size of the tumor.

Commonly, people with brain tumor experience long-term headaches, seizures or convulsions, difficulty thinking and speaking/finding words, personality changes, tingling or stiffness in one side of the body, a loss of balance, vision changes, nausea, and/or disorientation.

Consult with your doctor if you have any of the following symptoms:

  • Headaches that are new or worsening, especially in the morning or when lying down (these are often the first symptom of a brain tumor)
  • Frequent nausea or vomiting
  • Dizziness, loss of balance and coordination or room spinning
  • Vision, hearing, and speech problems
  • Difficulty with balance and trouble walking
  • Weakness on one side or part of the body
  • Unusual sleepiness or change in activity level
  • Changes in personality or mood, such as anger, irritability or emotional withdrawal
  • Changes to how you think
  • Inability to focus or vision loss
  • Confusion
  • Seizures
  • Loss of consciousness
  • Impaired sense of smell or taste
  • Drowsiness and fatigue
  • Endocrine dysfunction (hormone/gland changes)

You may notice other signs, like memory problems or difficulty speaking or remembering words.

Having one or more of the symptoms above does not necessarily mean that you have a brain or spinal cord tumor. All of these symptoms can have other causes. Still, if you have any of these symptoms, especially if they don’t go away or get worse over time, see your doctor so the cause can be found and treated, if needed.

Brain tumor causes

The cause of most brain tumors is unknown, but there are several risk factors that may increase your chances of developing a brain tumor. Researchers have also found some of the changes that occur in normal brain cells that may lead them to form brain tumors.

Normal human cells grow and function based mainly on the information in each cell’s DNA. Brain and spinal cord tumors, like other tumors, are caused by changes in the DNA inside cells. DNA is the chemical that makes up our genes, which control how our cells function. We usually look like our parents because they are the source of our DNA. But DNA affects more than how we look.

Some genes control when your cells grow, divide into new cells, and die:

  • Certain genes that help cells grow, divide, and stay alive are called oncogenes.
  • Genes that help keep cell division under control, repair mistakes in DNA, or make cells die at the right time are called tumor suppressor genes.

Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes. These gene changes can be inherited from a parent, but more often they happen during a person’s lifetime.

Inherited gene changes

Researchers have found gene changes that cause some rare inherited syndromes (like neurofibromatosis, tuberous sclerosis, Li-Fraumeni syndrome, and von Hippel-Lindau syndrome) that increase the risk of developing some brain and spinal cord tumors. For example, the Li-Fraumeni syndrome is caused by changes in the TP53 tumor suppressor gene. Normally, this gene prevents cells with damaged DNA from growing. Changes in this gene increase the risk of developing brain tumors (particularly gliomas), as well as some other cancers.

Gene changes acquired during a person’s lifetime

It’s usually not known why people without inherited syndromes develop brain or spinal cord tumors. Most exposures that cause cancer, such as chemicals in tobacco smoke, somehow damage DNA. But the brain is relatively protected from most cancer-causing chemicals that we might breathe in or eat, so these factors are not likely to play a major role in these cancers.

Several different gene changes usually occur in normal cells before they become cancerous. There are many kinds of brain tumors, each of which may have different sets of gene changes. A number of gene changes have been found in different brain tumor types, but there are probably many others that have not yet been found.

Researchers now understand some of the gene changes that occur in different types of brain tumors, but it’s still not clear what causes most of these changes. Some gene changes might be inherited, but most brain and spinal cord tumors are not the result of known inherited syndromes. Other than radiation, no known lifestyle-related or environmental factors are clearly linked to brain tumors. Most gene changes are probably just random events that sometimes happen inside a cell, without having an outside cause.

Risk factors for brain and spinal cord tumors

A risk factor is anything that increases your chances of getting a disease such as a brain or spinal cord tumor. Different types of cancer have different risk factors. But having a risk factor, or even several, does not always mean that a person will get the disease, and many people get brain or spinal cord tumors without having any known risk factors.

Many different types of tumors can start in the brain or spinal cord, and while they might have some things in common, these different tumors might not all have the same risk factors.

Most brain tumors are not linked with any known risk factors and have no obvious cause. But there are a few factors that can raise the risk of brain tumors.

Risk factors for brain and spinal cord tumors include:

  • Age – brain tumors can start at any age, but the risk of getting a brain tumor increases with age (most brain tumors happen in older adults aged between 85 and 89 years), although some types of brain tumor are more common in children
  • Radiation – exposure to radiation accounts for a very small number of brain tumors; some types of brain tumors are more common in people who have had radiotherapy, CT scans or X-rays of the head. Less than 1 out of every 100 brain tumors (less than 1%) diagnosed are caused by ionizing radiation. Most cases happen in people who have received radiation from previous radiotherapy treatments, rather than from x-rays and CT scans.
  • Having a weakened immune system. People with weakened immune systems have an increased risk of developing lymphomas of the brain or spinal cord (known as primary CNS lymphomas). Lymphomas are cancers of lymphocytes, a type of white blood cell that fights disease. Primary CNS lymphoma is less common than lymphoma that develops outside the brain. A weakened immune system can be congenital (present at birth), or it can be caused by treatments for other cancers, treatment to prevent rejection of transplanted organs, or diseases such as acquired immunodeficiency syndrome (AIDS).
  • Family history and genetic conditions – some genetic conditions are known to increase the risk of getting a brain tumor, including:
    • Neurofibromatosis (NF) type 1 and type 2
      • Neurofibromatosis type 1 (NF1) also known as von Recklinghausen disease, is genetic disorder that is the most common syndrome linked to brain or spinal cord tumors. People with neurofibromatosis type 1 (NF1) have higher risks of schwannomas, meningiomas, and certain types of gliomas, as well as neurofibromas (benign tumors of peripheral nerves). Changes in the NF1 gene cause neurofibromatosis type 1 (NF1). These changes are inherited from a parent in about half of all cases. In the other half, the NF1 gene changes occur before birth in people whose parents did not have this condition.
      • Neurofibromatosis type 2 (NF2) is much less common than neurofibromatosis type 1 (NF1), is associated with vestibular schwannomas (acoustic neuromas), which almost always occur on both sides of the head. Neurofibromatosis type 2 (NF2) is also linked with an increased risk of meningiomas or spinal cord ependymomas. Changes in the NF2 gene are usually responsible for neurofibromatosis type 2. Like neurofibromatosis type 1 (NF1), the gene changes are inherited in about half of cases. In the other half, they occur before birth in children without a family history.
    • Tuberous sclerosis (TSC). People with tuberous sclerosis may have subependymal giant cell astrocytomas (SEGAs), which are low-grade astrocytomas that develop beneath the ependymal cells of the ventricles. They may also have other benign tumors of the brain, skin, heart, kidneys, and other organs. Tuberous sclerosis (TSC) is caused by changes in either the TSC1 or the TSC2 gene. These gene changes can be inherited from a parent, but most often they develop in people without a family history.
    • Li-Fraumeni syndrome. People with Li-Fraumeni syndrome are at higher risk for developing gliomas, along with breast cancer, soft tissue sarcomas, leukemia, adrenal gland cancer, and certain other types of cancer. It is caused by changes in the TP53 gene.
    • Von Hippel-Lindau syndrome (VHL) also called VHL disease, VHL syndrome or von Hippel-Lindau disease. Von Hippel-Lindau syndrome is a rare, inherited disorder that causes tumors and cysts to grow in certain parts of the body, including the brain, spinal cord, eyes, inner ear, adrenal glands, pancreas, kidney, and reproductive tract. The tumors are usually benign (not cancer), but some may be malignant (cancer). Patients with von Hippel-Lindau syndrome have an increased risk of certain types of cancer, especially kidney cancer and pancreatic cancer. The signs and symptoms of von Hippel-Lindau syndrome usually do not appear until young adulthood. Von Hippel-Lindau syndrome is caused by a mutation (change) in the VHL gene. Most often the gene changes are inherited, but in some cases the changes happen before birth in people whose parents don’t have them.
    • Turner syndrome. Turner syndrome (45X) is a genetic condition that occurs in females who have a missing or abnormal X chromosome (sex chromosome). Turner syndrome is marked by a shorter-than-average height, ovaries that do not function normally, and infertility. Other signs and symptoms may include extra folds of skin on the neck, a low hairline at the back of the neck, swelling of the hands and feet, and heart, kidney, bone, and hearing problems. Turner syndrome is present at birth but its signs and symptoms may occur slowly over time. Having Turner syndrome increases the risk of germ cell tumors in the ovaries.
    • Turcot syndrome. Turcot syndrome also known as brain tumor-polyposis syndrome, describes people who have many colon polyps and an increased risk of colorectal cancer, as well as an increased risk for certain types of brain tumors. But this syndrome is actually made up of two different hereditary conditions:
      • When linked with familial adenomatous polyposis (FAP), there is a mutation in the APC gene. In people with this gene mutation, brain tumors are typically medulloblastomas.
      • When linked with Lynch syndrome (also known as hereditary non-polyposis colorectal cancer or HNPCC), the mutation is in one of the mismatch repair genes, such as MLH1 or PMS2. In people with one of these gene mutations, brain tumors are usually gliomas.
    • Gorlin syndrome also called basal cell nevus syndrome, BCNS, NBCCS or nevoid basal cell carcinoma syndrome. Gorlin syndrome is a rare, inherited disorder that affects many organs and tissues in the body. People with Gorlin syndrome have a very high risk of developing basal cell skin cancer during adolescence or early adulthood. They are also at risk of developing medulloblastoma (a type of brain cancer) and other types of cancer. Gorlin syndrome may also cause benign (not cancer) tumors in the jaw, heart, or ovaries. Other signs and symptoms include a large head and unusual facial features; small pits in the skin on the hands and feet; abnormalities of the spine, ribs, or skull; eye problems; and developmental problems. Gorlin syndrome is caused by a mutation (change) in the PTCH1 gene.
    • Cowden syndrome also called Cowden disease or multiple hamartoma syndrome, is a rare inherited disorder marked by the presence of many benign (not cancer) growths called hamartomas and an increased risk of developing certain cancers. Hamartomas form in different parts of the body, especially the skin, mouth, and gastrointestinal tract. Other benign tumors may also occur in the thyroid, breast, uterus, soft tissue, and brain. Other signs and symptoms include a larger-than-average head, abnormal skin changes, blood vessel problems, autism spectrum disorder, and learning and developmental delays. People with Cowden syndrome have an increased risk of developing certain types of cancer, including melanoma and cancers of the breast, thyroid, endometrium, kidney, colon, and rectum. Cowden syndrome is usually caused by mutations (changes) in the PTEN gene.

Controversial risk factors

Cell phone use

Cell phones give off radiofrequency (RF) rays, a form of energy on the electromagnetic spectrum between FM radio waves and those used in microwave ovens, radar, and satellite stations. Cell phones do not give off ionizing radiation, the type that can cause cancer by damaging the DNA inside cells. Still, there have been concerns that the phones, whose antennae are built-in and therefore are placed close to the head when being used, might somehow raise the risk of brain tumors.

Some studies have suggested a possible increased risk of brain tumors or of vestibular schwannomas (acoustic neuromas) with cell phone use, but most of the larger studies done so far have not found an increased risk, either overall or among specific types of tumors. Still, there are very few studies of long-term use (10 years or more), and cell phones haven’t been around long enough to determine the possible risks of lifetime use. The same is true of any possible higher risks in children, who are increasingly using cell phones. Cell phone technology also continues to change, and it’s not clear how this might affect any risk.

These risks are being studied, but it will probably be many years before firm conclusions can be made. In the meantime, for people concerned about the possible risks, there are ways to lower your exposure, such as using the phone’s speaker or an earpiece to move the phone itself away from the head.

Environmental factors

Exposure to vinyl chloride (a chemical used to manufacture plastics), petroleum products, and certain other chemicals have been linked with an increased risk of brain tumors in some studies but not in others.

Exposure to aspartame (a sugar substitute), exposure to electromagnetic fields from power lines and transformers, and infection with certain viruses have been suggested as possible risk factors, but most researchers agree that there is no convincing evidence to link these factors to brain tumors. Research on these and other possible risk factors continues.

Brain tumor prevention

The risk of many cancers in adults can be reduced with certain lifestyle changes (such as quitting smoking). But other than radiation exposure, there are no known lifestyle-related or environmental risk factors for brain and spinal cord tumors, so at this time there is no known way to protect against most of these tumors.

For most people with other types of cancer in or near the head, radiation therapy may be given if doctors feel the benefits of the treatment outweigh the small risk of developing a brain tumor years later. Still, when it is needed, doctors try to limit the dose of radiation as much as possible.

Imaging tests such as x-rays or CT scans use much lower levels of radiation than those used for cancer treatment. If there is any increase in risk from these tests, it is likely to be very small, but to be safe, most doctors recommend that people (especially pregnant women and children) not get these tests unless they are absolutely needed.

Can brain and spinal cord tumors in adults be found early?

Screening is testing for a disease in people who have no symptoms. At this time there are no widely recommended tests to screen for brain and spinal cord tumors. Most brain tumors are found when a person goes to a doctor because of signs or symptoms they are having.

For people with certain inherited syndromes (such as neurofibromatosis or tuberous sclerosis) that put them at higher risk for brain tumors, doctors often recommend frequent physical exams and other tests starting when they are young. In some cases these tests can find tumors when they are still small. Not all tumors related to these syndromes may need to be treated right away, but finding them early might help doctors monitor them so that they can be treated quickly if they begin to grow or cause problems.

Brain tumor diagnosis

Brain and spinal cord tumors are usually found because of signs or symptoms a person is having. If it’s suspected that you have a brain tumor, your doctor may recommend a number of tests and procedures, including:

  • A neurological exam. A neurological exam may include, among other things, checking your vision, hearing, balance, coordination, strength and reflexes. Difficulty in one or more areas may provide clues about the part of your brain that could be affected by a brain tumor.
  • Imaging tests. Magnetic resonance imaging (MRI) and computed tomography (CT) scans are used most often to look for brain diseases. These scans will almost always show a brain tumor, if one is present. Doctors can often also get an idea about what type of tumor it might be, based on how it looks on the scan and where it is in the brain.
    • Magnetic resonance imaging (MRI) scan. MRI scans use radio waves and strong magnets (instead of x-rays) to make pictures. A contrast material called gadolinium may be injected into a vein before the scan to help see details better. MRI scans are very good for looking at the brain and spinal cord and are considered the best way to look for tumors in these areas. The images they provide are usually more detailed than those from CT scans. But they do not pick up the bones of the skull as well as CT scans and therefore may not show the effects of tumors on the skull.
      • Special types of MRI can be useful in some situations:
        • Magnetic resonance angiography (MRA) and magnetic resonance venography (MRV): These special types of MRI may be used to look at the blood vessels in the brain. This can be very useful before surgery to help the surgeon plan an operation.
        • Magnetic resonance spectroscopy (MRS): This test can be done as part of an MRI. It measures biochemical changes in an area of the brain (displayed in graph-like results called spectra, although basic images can also be created). By comparing the results for a tumor to that of normal brain tissue, it can sometimes help determine the type of tumor (or how quickly it is likely to grow), although a biopsy of the tumor is often still needed to get an accurate diagnosis. Magnetic resonance spectroscopy (MRS) can also be used after treatment to help determine if an area that still looks abnormal on another test is remaining tumor or if it is more likely to be scar tissue.
        • Magnetic resonance perfusion: For this test, also known as perfusion MRI, a contrast dye is injected quickly into a vein. A special type of MR image is then obtained to look at the amount of blood going through different parts of the brain and tumor. Tumors often have a bigger blood supply than normal areas of the brain. A faster growing tumor may need more blood. Perfusion MRI can give doctors an idea of the best place to take a biopsy. It can also be used after treatment to help determine if an area that still looks abnormal is remaining tumor or if it is more likely to be scar tissue.
        • Functional MRI (fMRI): This test looks for tiny blood flow changes in an active part of the brain. It can be used to determine what part of the brain handles a function such as speech, thought, sensation, or movement. Doctors can use this to help determine which parts of the brain to avoid when planning surgery or radiation therapy. This test is similar to a standard MRI, except that you will be asked to do specific tasks (such as answering simple questions or moving your fingers) while the scans are being done.
    • Computed tomography (CT) scan. A CT scan uses x-rays to make detailed cross-sectional images of your brain and spinal cord (or other parts of the body). Unlike a regular x-ray, a CT scan creates detailed images of the soft tissues in the body. CT scans are not used as often as MRI scans when looking at brain or spinal cord tumors, but they can be useful in some cases. They may be used if MRI is not an option (such as in people who are very overweight or people who have a fear of enclosed spaces). CT scans also show greater detail of the bone structures near the tumor. As with MRI, you may get an injection of a contrast dye through an IV (intravenous) line before the scan (although a different dye is used for CT scans). This helps better outline any tumors that are present.
      • CT angiography (CTA): For this test, you are injected with a contrast material through an IV line while you are in the CT scanner. The scan creates detailed images of the blood vessels in the brain, which can help doctors plan surgery. CT angiography can provide better details of the blood vessels in and around a tumor than MR angiography in some cases.
    • Positron emission tomography (PET) scan. For a PET scan, you are injected with a slightly radioactive substance (usually a type of sugar known as FDG) which collects mainly in tumor cells. A special camera is then used to create a picture of areas of radioactivity in the body. The picture is not as detailed as a CT or MRI scan, but it can provide helpful information about whether abnormal areas seen on other tests (such as MRIs) are likely to be tumors or not. This test is more likely to be helpful for fast-growing (high-grade tumors) than for slower-growing tumors. This test is also useful after treatment to help determine if an area that still looks abnormal on an MRI scan is remaining tumor or if it is more likely to be scar tissue. Remaining tumor might show up on the PET scan, while scar tissue will not.
    • Chest x-ray. A chest x-ray might be done to look for tumors in the lungs if a tumor is found in the brain. This is because in adults, most tumors in the brain actually have started in another organ (most often the lung) and then spread to the brain. This test can be done in a doctor’s office, in an outpatient radiology center, or in a hospital.
  • Brain or spinal cord tumor biopsy. Imaging tests such as MRI and CT scans may show an abnormal area that is likely to be a brain or spinal cord tumor. But these scans can’t always tell exactly what type of tumor it is. Often this can only be done by removing some of the tumor tissue in a procedure called a biopsy. A biopsy may be done as a procedure on its own, or it may be part of surgery to remove the tumor. Sometimes, a tumor may look so characteristically obvious on an MRI scan (for example, clearly looking like an astrocytoma) that a biopsy is not needed, especially if the tumor is in a part of the brain that would make it hard to biopsy (such as the brain stem). In rare cases a PET scan or MR spectroscopy may give enough information so that a biopsy is not needed. The 2 main types of biopsies for brain tumors are:
    • Stereotactic (needle) biopsy. Stereotactic (needle) biopsy may be used if, based on imaging tests, surgery to remove the tumor might be too risky (such as with some tumors in vital areas, those deep within the brain, or other tumors that probably can’t be removed safely with surgery) but a sample is still needed to make a diagnosis. The patient may be asleep (under general anesthesia) or awake during the biopsy. If the patient is awake, the neurosurgeon injects a local anesthetic into areas of skin above the skull to numb them. The skull and brain do not feel pain. The biopsy itself can be done in two main ways:
      • One approach is to get an MRI or CT, and then use either markers (each about the size of a nickel) placed on different parts of the scalp, or facial and scalp contours, to create a map of the inside of the head. An incision (cut) is then made in the scalp, and a small hole is drilled in the skull. An image-guidance system is then used to direct a hollow needle into the tumor to remove small pieces of tissue.
      • In another approach that’s being used less often, a rigid frame is attached to the head. An MRI or CT scan is often used along with the frame to help the neurosurgeon guide a hollow needle into the tumor. This also requires an incision in the scalp and a small hole in the skull.
      • The removed tissue is sent to a pathologist (a doctor specializing in diagnosis of diseases by lab tests). Sometimes it might need to be looked at by a neuropathologist, a pathologist who specializes in nervous system diseases. The pathologist looks at it under a microscope (and might do other lab tests) to determine if the tumor is benign or malignant (cancerous) and exactly what type of tumor it is. This is very important in determining a person’s prognosis (outlook) and the best course of treatment. A preliminary diagnosis might be available the same day, although it often takes at least a few days to get a final diagnosis.
    • Surgical or open biopsy (craniotomy). If imaging tests show the tumor can likely be treated with surgery, the neurosurgeon may not do a needle biopsy. Instead, an operation called a craniotomy might be done to remove all or most of the tumor. (If removing all of the tumor would likely damage nearby important structures, removing most of the tumor, known as debulking, might be done.) For a preliminary diagnosis, small samples of the tumor are looked at right away by the pathologist while the patient is still in the operating room. This can help guide treatment, including whether further surgery should be done at that time. A final diagnosis is made within a few days in most cases.
  • Lab tests of biopsy specimens. Finding out which type of tumor someone has is very important in helping to determine their outlook (prognosis) and treatment options. But in recent years, doctors have found that changes in certain genes, chromosomes, or proteins within the cancer cells can also be important. Some tumors are now tested for these types of changes. For example:
    • Gliomas that are found to have IDH1 or IDH2 gene mutations tend to have a better outlook than gliomas without these gene mutations.
    • In high-grade gliomas, the presence of MGMT promoter methylation is linked with better outcomes and a higher likelihood of responding to chemotherapy.
    • Lab tests looking for other gene or chromosome changes might also be done.
  • Lumbar puncture (spinal tap). Lumbar puncture (spinal tap) is used mainly to look for cancer cells in the cerebrospinal fluid (CSF), the liquid that surrounds the brain and spinal cord. For this test, you lie on your side on a bed or exam table with your knees up near your chest. The doctor first numbs an area in the lower part of the back near the spine. A small, hollow needle is then placed between the bones of the spine to withdraw some of the fluid. This fluid is sent to a lab to be looked at for cancer cells. Other tests may be done on the fluid as well. Lumbar punctures are usually very safe, but doctors have to make sure the test does not result in a large drop in fluid pressure inside the skull, which could possibly cause serious problems. For this reason, imaging tests such as CT or MRI scans are done first. Lumbar punctures usually aren’t done to diagnose brain tumors, but they may be done to help determine the extent of a tumor by looking for cancer cells in the CSF. They are often used if a tumor has already been diagnosed as a type that can commonly spread through the CSF, such as an ependymoma. Lumbar punctures are particularly important in people with suspected brain lymphomas because lymphoma cells often spread into the CSF.
  • Blood and urine tests. Blood and urine tests rarely are part of the actual diagnosis of brain and spinal cord tumors, but they may be done to check how well the liver, kidneys, and some other organs are working. This is especially important before any planned surgery. If you are getting chemotherapy, blood tests will be done routinely to check blood counts and to see if the treatment is affecting other parts of your body.

If you’re uncertain about your diagnosis, consider seeking a second opinion at a medical center where many brain biopsies are evaluated every year.

Brain and spinal cord tumors prognostic factors

Several factors are important when doctors are trying to figure out how best to treat a brain or spinal cord tumor and what the prognosis (outlook) is likely to be:

  • The type of tumor (based on the type of cell it starts in): Tumors can form in almost any type of tissue or cell in the brain or spinal cord. Some tumors have a mix of cell types. Different types of tumors tend to start in certain parts of the brain or spinal cord, and tend to grow in certain ways. (The most common types of brain and spinal cord tumors in adults are described below.)
  • The grade of the tumor: Some types of brain and spinal cord tumors are more likely to grow into nearby brain or spinal cord tissue (and to grow quickly) than are others. Brain and spinal cord tumors are typically divided into 4 grades (using Roman numerals I to IV), based largely on how the tumor cells look under a microscope:
    • Lower grade (grade 1 or 2) tumors tend to grow more slowly and are less likely to grow into (invade or infiltrate) nearby tissues.
      • Grade 1 the cells look very like normal cells. They are usually slow growing and less likely to spread. Surgery is usually the only treatment you need for a grade 1 brain tumor.
      • Grade 2 the cells look less like normal cells. They are usually slow growing but can grow into the nearby brain tissue. Grade 2 tumors are more likely to come back after surgery and some can develop into a malignant tumor.
    • Higher grade (grade 3 or 4) tumors tend to grow quickly and are more likely to grow into nearby tissues. These tumors often require more intense treatment.
      • Grade 3 the cells look more abnormal. They can spread to other parts of the brain and the spinal cord. You are more likely to need radiotherapy and chemotherapy after surgery.
      • Grade 4 the cells look very abnormal. These are the fastest growing tumors. They often come back after treatment and can spread to other parts of the brain and sometimes the spinal cord. You usually have treatment with radiotherapy and chemotherapy.
  • Changing from benign to malignant. Some low grade tumors can develop into a malignant tumor. It is called malignant transformation or progression to malignancy. For example, a grade 2 tumor could progress to a grade 3 tumor. Or a grade 3 tumor could change to a grade 4.
  • Gene changes in the tumor cells: Even for a specific type of brain tumor, the changes in the genes (DNA) of the tumor cells can be different. For example, many types of tumors are now divided based on whether the cells have mutations in one of the isocitrate dehydrogenase (IDH) genes (IDH1 or IDH2). For a specific type of tumor, those with IDH mutations tend to have a better outlook than those without a mutation. Other gene mutations can also be important for certain types of tumors. For example, for a type of brain tumour called glioma, you might have tests to look at proteins called: IDH, 1p/19q and MGMT.
  • The location of the tumor: Where the tumor is in the brain or spinal cord can affect what symptoms it causes, as well as which treatments might be best.

Brain tumor treatment

If you have a brain tumor, your treatment will depend on:

  • the type of tumor
  • where it is in your brain
  • how big it is and how far it’s spread
  • how abnormal the cells are
  • your overall health and fitness

Treatments for brain tumors include:

  • steroids
  • medicines to help with symptoms
  • surgery
  • radiotherapy
  • chemotherapy
  • targeted drug therapy
  • alternating electric field therapy

After being diagnosed with a brain tumor, steroids may be prescribed to help reduce swelling around the tumor. Other medicines can be used to help with other symptoms of brain tumors, such as anti-epileptic medicines for seizures and painkillers for headaches.

Surgery is often used to remove brain tumors. The aim is to remove as much abnormal tissue as safely as possible.

It’s not always possible to remove all of a tumor, so further treatment with radiotherapy or chemotherapy may be needed to treat any abnormal cells left behind.

Treatment for non-cancerous tumors is often successful and a full recovery is possible.

Sometimes there’s a small chance the tumor could return, so you may need regular follow-up appointments to monitor this.

Corticosteroids

Corticosteroid drugs such as dexamethasone (Decadron) are often given to reduce swelling around brain tumors. This may help relieve headaches and other symptoms.

Medicines to help with symptoms

Some drugs commonly used in people with brain or spinal cord tumors do not treat the tumors directly, but they may help lessen symptoms caused by the tumor or its treatment.

Anti-seizure drugs (anticonvulsants)

Drugs may also be given to lower the chance of seizures in people with brain tumors. Different anti-seizure drugs can be used. Because many of these drugs can affect how other drugs such as chemotherapy work in the body, they are not usually given unless the tumor has caused seizures.

Hormones

The pituitary gland helps control the levels of many different hormones in the body. If the pituitary gland is damaged by the tumor itself or by tumor treatments (such as surgery or radiation therapy), you may need to take pituitary hormones or other hormones to replace those missing.

Brain tumor surgery

Surgery on brain and spinal cord tumors may be done to:

  • Get a biopsy sample to determine the type of tumor
  • Remove the tumor (or as much of it as possible)
  • Help prevent or treat symptoms or possible complications from the tumor

Before surgery, be sure you understand the goal of the surgery, as well as its possible benefits and risks.

Surgery on the brain or spinal cord is a serious operation, and surgeons are very careful to try to limit any problems either during or after surgery. Complications during or after any type of surgery can include bleeding, infections, or reactions to anesthesia, although these are not common.

A major concern after surgery is swelling in the brain. Drugs called corticosteroids are typically given before and for several days after surgery to help lessen this risk.

Seizures are also possible after brain surgery. Anti-seizure medicines can help lower this risk, although they might not prevent them completely.

One of the biggest concerns when removing brain tumors is the possible loss of brain function afterward, which is why doctors are very careful to remove only as much tissue as is safely possible. If problems do arise, it could be right after surgery, or it could be days or even weeks later, so close monitoring for any changes is very important.

Surgery to remove the tumor

Most often, the first step in brain or spinal cord tumor treatment is for the neurosurgeon to remove as much of the tumor as is safe without affecting normal brain function. Surgery alone or combined with radiation therapy may control or cure many types of tumors, including some low-grade astrocytomas, ependymomas, craniopharyngiomas, gangliogliomas, and meningiomas.

Tumors that tend to spread widely into nearby brain or spinal cord tissue, such as anaplastic astrocytomas or glioblastomas, typically cannot be cured by surgery. But surgery is often done first to reduce the amount of tumor that needs to be treated by radiation or chemotherapy, which might help these treatments work better. This could help prolong the person’s life, even if all of the tumor can’t be removed.

Surgery can also be done to help relieve some of the symptoms caused by brain tumors, particularly those caused by a buildup of pressure within the skull. These can include headaches, nausea, vomiting, and blurred vision. Surgery may also make seizures easier to control with medicines.

Surgery to remove the tumor may not be a good option in some situations, such as if the tumor is deep within the brain, if it’s in a part of the brain that can’t be removed, such as the brain stem, or if a person can’t have a major operation for other health reasons.

Surgery is not very effective against some types of brain tumors, such as lymphomas, although it may be used to get a biopsy sample for diagnosis.

Craniotomy

A craniotomy is a surgical opening made in the skull. This is the most common approach for surgery to treat brain tumors. The person may either be under general anesthesia (in a deep sleep) or may be awake for at least part of the procedure (with the surgical area numbed) if brain function needs to be assessed during the operation.

Part of the head might be shaved before surgery. The neurosurgeon first makes a cut in the scalp over the skull near the tumor, and folds back the skin. A special type of drill is used to remove the piece of the skull over the tumor.

The opening is typically large enough for the surgeon to insert several instruments and see the parts of the brain needed to operate safely. The surgeon may need to cut into the brain itself to reach the tumor. The surgeon might use MRI or CT scans taken before the surgery (or may use ultrasound once the skull has been opened) to help locate the tumor and its edges.

The surgeon can remove the tumor in different ways depending on how hard or soft it is, and whether it has many or just a few blood vessels:

  • Many tumors can be cut out with a scalpel or special scissors.
  • Some tumors are soft and can be removed with suction devices.
  • In other cases, a handheld ultrasonic aspirator can be placed into the tumor to break it up and suck it out.

Many devices can help the surgeon see the tumor and surrounding brain tissue. The surgeon often operates while looking at the brain through a special microscope. MRI or CT scans can be done before surgery (or ultrasound can be used once the skull has been opened) to map the area of tumors deep in the brain. In some cases, the surgeon uses intraoperative imaging, in which MRI (or other) images are taken at different times during the operation to show the location of any remaining tumor. This may allow some brain tumors to be resected more safely and extensively.

As much of the tumor is removed as possible while trying not to affect brain functions. The surgeon can use different techniques to lower the risk of removing vital parts of the brain, such as:

  • Intraoperative cortical stimulation (cortical mapping): In this approach, the surgeon electrically stimulates parts of the brain in and around the tumor during the operation and monitors the response. This can show if these areas control an important function (and therefore should be avoided).
  • Functional MRI: This type of imaging test can be done before surgery to locate a particular function of the brain. This information can be used to identify and preserve that region during the operation.
  • Fluorescence-guided surgery: For some types of tumors, such as glioblastomas, the patient can be given a special fluorescent dye before surgery. The dye is taken up by the tumor, which then glows when the surgeon looks at it under fluorescent lighting from the operating microscope. This lets the surgeon better separate tumor from normal brain tissue.
  • Newer techniques: Newer types of MRI, as well as newer surgical approaches, might be helpful in some situations.

Once the surgery is complete, the piece of the skull bone is put back in place and fastened with metal screws and plates, wires, or special stitches. Usually any metal pieces are made from titanium, which allows a person to get follow-up MRIs [and will not set off metal detectors].

You might have small tube (called a drain) coming out of the incision that allows excess cerebrospinal fluid (CSF) to leave the skull. Other drains may be in place to allow blood that builds up after surgery to drain from under the scalp. These drains are usually removed after a few days. An imaging test such as an MRI or CT scan is typically done 1 to 3 days after the operation to confirm how much of the tumor has been removed. Recovery time in the hospital is usually 4 to 6 days, although this depends on the size and location of the tumor, the patient’s general health, and whether other treatments are given. Healing around the surgery site usually takes several weeks.

Surgery to help with CSF flow blockage

If a tumor blocks the flow of cerebrospinal fluid (CSF), it can increase pressure inside the skull (known as increased intracranial pressure, or ICP). This can cause symptoms like headaches, nausea, and drowsiness, and may even be life-threatening. Surgery to remove the tumor can often help with this, but there are also other ways to drain away excess CSF and lower the pressure if needed.

For example, the neurosurgeon may put in a silicone tube called a shunt (also called a ventriculoperitoneal shunt or VP shunt). One end of the shunt is placed in a ventricle of the brain (an area filled with CSF) and the other end is placed in the abdomen or, less often, the heart (and would then be referred to as a ventriculoatrial shunt). The tube runs under the skin of the neck and chest. The flow of CSF is controlled by a valve placed along the tubing.

Shunts can be temporary or permanent. They can be placed before or after the surgery to remove the tumor. Placing a shunt normally takes about an hour. As with any operation, complications might develop, such as bleeding or infection. Strokes are possible as well. Sometimes shunts get clogged and need to be replaced. The hospital stay after shunt procedures is typically 1 to 3 days, depending on the reason it is placed and the patient’s general health.

Another option to treat increased pressure in the skull in some cases is an endoscopic third ventriculostomy (ETV). In this operation, an opening is made in the floor of the third ventricle at the base of the brain to allow the CSF to flow again. This operation is done through a small hole in the front of the skull. An advantage of this approach is that it does not require a shunt. But there is also a chance that the opening made in the ventricle might close up again, which is more likely in people with brain tumors.

If the pressure inside the head needs to be relieved for a short time, an external ventricular drain (EVD) might be put in place to allow the excess CSF to drain out. The drain is a small tube. One end is put into a ventricle, and the other end is attached to a collection bag outside the body. Along with collecting the excess CSF, the drain can also be used to measure the pressure inside the skull, as well as to look for tumor cells, blood, or signs of infection in the CSF.

The drain can be placed either during surgery or during a procedure at the patient’s bedside. It can be put in place to relieve the pressure in the days before surgery, or to help drain the fluid that collects after an operation. If the pressure inside the skull needs to be lowered for more than a few days, the doctor might need to change this to a VP shunt.

Surgery to put in a ventricular access catheter

Surgery may also be used to insert a ventricular access catheter, such as an Ommaya reservoir, to help deliver chemotherapy directly into the CSF. A small incision is made in the scalp, and a small hole is drilled in the skull. A flexible tube is then threaded through the hole until the open end of the tube is in a ventricle, where it reaches the CSF. The other end, which has a dome-shaped reservoir, stays just under the scalp. After the operation, doctors and nurses can use a thin needle to give chemotherapy drugs through the reservoir or to remove CSF from the ventricle for testing.

Radiation therapy

Radiation therapy uses high-energy beams, such as X-rays or protons, to kill tumor cells. Radiation therapy can come from a machine outside your body (external beam radiation), or, very rarely, radiation can be placed inside your body close to your brain tumor (brachytherapy).

External beam radiation can focus just on the area of your brain where the tumor is located, or it can be applied to your entire brain (whole-brain radiation). Whole-brain radiation is most often used to treat cancer that spreads to the brain from some other part of the body and forms multiple tumors in the brain.

Traditionally, radiation therapy uses X-rays, but a newer form of this treatment uses proton beams. Proton beam therapy allows doctors to control the radiation more precisely. It may be helpful for treating brain tumors in children and tumors that are very close to sensitive areas of the brain. Proton beam therapy isn’t as widely available as traditional X-ray radiation therapy.

Before your treatments start, the radiation team will determine the correct angles for aiming the radiation beams and the proper dose of radiation. This planning session, called simulation, usually includes getting imaging tests such as CT or MRI scans.

In most cases, the total dose of radiation is divided into daily amounts (usually given Monday through Friday) over several weeks. At each treatment session, you lie on a special table while a machine delivers the radiation from precise angles. The treatment is not painful. Each session lasts about 15 to 30 minutes, and much of that time is spent making sure the radiation is aimed correctly. The actual treatment time each day is much shorter.

High doses of radiation therapy can damage normal brain tissue, so doctors try to deliver the radiation to the tumor while giving the lowest possible dose to normal surrounding brain areas. Several techniques can help doctors focus the radiation more precisely:

  • Three-dimensional conformal radiation therapy (3D-CRT): 3D-CRT uses the results of imaging tests such as MRI and special computers to map the location of the tumor precisely. Several radiation beams are then shaped and aimed at the tumor from different directions. Each beam alone is fairly weak, which makes it less likely to damage normal tissues, but the beams converge at the tumor to give a higher dose of radiation there.
  • Intensity modulated radiation therapy (IMRT): Intensity modulated radiation therapy (IMRT): is an advanced form of 3D therapy. It uses a computer-driven machine that moves around the patient as it delivers radiation. Along with shaping the beams and aiming them at the tumor from several angles, the intensity (strength) of the beams can be adjusted to limit the dose reaching the most sensitive normal tissues. This may let the doctor deliver a higher dose to the tumor. Many major hospitals and cancer centers now use IMRT.
  • Volumetric modulated arc therapy (VMAT): This newer technique is similar to IMRT. For this treatment, the patient lies on a table, which passes through the machine delivering the radiation. The source of the radiation (the linear accelerator) rotates around the table in an arc, delivering the beams from different angles. A computer controls the intensity of the beams to help keep the radiation focused on the tumor. It’s not yet clear if this approach results in better outcomes than IMRT, although it does allow the radiation to be given over less time in each treatment session.
  • Conformal proton beam radiation therapy: Proton beam therapy uses an approach similar to 3D-CRT. But instead of using x-rays, it focuses proton beams on the tumor. Protons are positive parts of atoms. Unlike x-rays, which release energy both before and after they hit their target, protons cause little damage to tissues they pass through and then release their energy after traveling a certain distance. This lets doctors deliver more radiation to the tumor and do less damage to nearby normal tissues. This approach may be more helpful for brain tumors that have distinct edges (such as chordomas), but it is not clear if it will be useful for tumors that typically grow into or mix with normal brain tissue (such as astrocytomas or glioblastomas). There are a limited number of proton beam centers in the United States at this time.
  • Stereotactic radiosurgery (SRS)/stereotactic radiotherapy (SRT): Stereotactic radiosurgery is not a form of surgery in the traditional sense. (There is no actual surgery in this treatment.) Instead, radiosurgery uses multiple beams of radiation to give a highly focused form of radiation treatment to kill the tumor cells in a very small area. Each beam of radiation isn’t particularly powerful, but the point where all the beams meet — at the brain tumor — receives a very large dose of radiation to kill the tumor cells. Stereotactic radiosurgery (SRS)/stereotactic radiotherapy (SRT) delivers a large, precise radiation dose to the tumor area in a single session (stereotactic radiosurgery [SRS]) or in a few sessions (stereotactic radiotherapy [SRT]). It may be used for some tumors in parts of the brain or spinal cord that can’t be treated with surgery or when a patient isn’t healthy enough for surgery. A head frame might be attached to the skull to help aim the radiation beams. (Sometimes a face mask is used to hold the head in place instead.) Once the exact location of the tumor is known from CT or MRI scans, radiation is focused at the tumor from many different angles. This can be done in 2 ways:
    • In one approach, thin radiation beams are focused at the tumor from hundreds of different angles for a short period of time. Each beam alone is weak, but they all converge at the tumor to give a higher dose of radiation. An example of a machine that uses this technique is the Gamma Knife.
    • Another approach uses a movable linear accelerator (a machine that creates radiation) that is controlled by a computer. Instead of delivering many beams at once, this machine moves around the head to deliver radiation to the tumor from many different angles. Several machines with names such as X-Knife, CyberKnife, and Clinac deliver stereotactic radiosurgery in this way.
    • Stereotactic radiosurgery (SRS) typically delivers the whole radiation dose in a single session, though it may be repeated if needed.
    • For stereotactic radiotherapy (SRT) (also called fractionated radiosurgery), doctors give the radiation in several treatments to deliver the same or a slightly higher dose. Frameless techniques are now available to make this more comfortable.
  • Image-guided radiation therapy (IGRT): For image-guided radiation therapy (IGRT), an imaging test such as a CT scan is done just before each treatment to help better guide the radiation to its target. Image-guided radiation therapy (IGRT) is typically used along with some of the more precise techniques for delivering radiation described above. It is most useful when the radiation needs to be delivered very precisely, such as when a tumor is very close to vital structures.
  • Brachytherapy (internal radiation therapy): Unlike the external radiation approaches above, brachytherapy involves inserting radioactive material directly into or near the tumor. The radiation it gives off travels a very short distance, so it affects only the tumor. This technique is most often used along with external radiation. It provides a high dose of radiation at the tumor site, while the external radiation treats nearby areas with a lower dose.
  • Whole brain and spinal cord radiation therapy (craniospinal radiation): If tests like an MRI scan or lumbar puncture find the tumor has spread along the covering of the spinal cord (meninges) or into the surrounding cerebrospinal fluid, radiation may be given to the whole brain and spinal cord. Some tumors such as ependymomas and medulloblastomas are more likely to spread this way and often require craniospinal radiation.

Radiation is more harmful to tumor cells than it is to normal cells. Still, radiation can also damage normal brain tissue, which can lead to side effects. Side effects of radiation therapy depend on the type and dose of radiation you receive. Common side effects during or immediately following radiation include fatigue, headaches, memory loss, scalp irritation and hair loss.

  • Side effects during or soon after treatment: Some people become irritable and tired during the course of radiation therapy. Nausea, vomiting, and headaches are also possible side effects but are uncommon. Sometimes dexamethasone (a corticosteroid) or other drugs can help relieve these symptoms. Some people might have hair loss in areas of the scalp that get radiation. Other side effects are also possible, depending on where the radiation is aimed.
  • Problems with thinking and memory: A person may lose some brain function if large areas of the brain get radiation. Problems can include memory loss, personality changes, and trouble concentrating. There may also be other symptoms depending on the area of brain treated and how much radiation was given. These risks must be balanced against the risks of not using radiation and having less control of the tumor.
  • Radiation necrosis: Rarely after radiation therapy, a mass of dead (necrotic) tissue forms at the site of the tumor in the months or years after radiation treatment. This can often be controlled with corticosteroid drugs, but surgery may be needed to remove the necrotic tissue in some instances.
  • Increased risk of another tumor: Radiation can damage genes in normal cells. As a result, there is a small risk of developing a second cancer in an area that got radiation — for example, a meningioma of the coverings of the brain, another brain tumor, or less likely a bone cancer in the skull. If this develops, it’s usually many years after the radiation is given. This small risk should not prevent those who need radiation from getting treatment.

Chemotherapy

Chemotherapy (chemo) uses anti-cancer drugs to kill tumor cells. Chemotherapy drugs can be taken orally in pill form or injected into a vein (intravenously). However, many chemo drugs aren’t able to enter the brain and reach tumor cells. For some brain tumors, drugs can be given directly into the cerebrospinal fluid (CSF, the fluid that bathes the brain and spinal cord), either in the brain or into the spinal canal below the spinal cord. To help with this, a thin tube known as a ventricular access catheter may be inserted through a small hole in the skull and into a ventricle of the brain during a minor operation.

In general, chemo is used for faster-growing brain tumors. Some types of brain tumors, such as medulloblastoma and lymphoma, tend to respond better to chemo than others. Chemo is not as helpful for treating some other types of tumors, such as spinal cord tumors, so it is used less often for these tumors.

Chemo is most often used along with other treatments such as surgery and/or radiation therapy. Chemo can also be used by itself, especially for more advanced tumors or for tumors that have come back after other types of treatment.

Some of the chemo drugs used to treat brain and spinal cord tumors include:

  • Carboplatin
  • Carmustine (BCNU)
  • Cisplatin
  • Cyclophosphamide
  • Etoposide
  • Irinotecan
  • Lomustine (CCNU)
  • Methotrexate
  • Procarbazine
  • Temozolomide (Temodar)
  • Vincristine

These drugs can be used alone or in combinations, depending on the type of brain tumor. Tests of your brain tumor cells can determine whether chemotherapy will be helpful for you. The type of brain tumor you have also is helpful in determining whether to recommend chemotherapy.

Chemotherapy is given in cycles, with each period of treatment followed by a rest period to give the body time to recover. Each cycle typically lasts for a few weeks.

Carmustine (Gliadel) wafers: These dissolvable wafers contain the chemo drug carmustine (BCNU). After the surgeon removes as much of the brain tumor as is safe during a craniotomy, the wafers can be placed directly on or next to the parts of the tumor that can’t be removed. Unlike IV or oral chemo that reaches all areas of the body, this type of therapy concentrates the drug at the tumor site, producing few side effects in other parts of the body.

Chemo drugs can cause side effects and the side effects depend on the type and dose of drugs you receive including and how long treatment lasts.

Common chemo side effects can include:

  • Hair loss
  • Mouth sores
  • Loss of appetite
  • Nausea and vomiting
  • Diarrhea
  • Increased chance of infections (from having too few white blood cells)
  • Easy bruising or bleeding (from having too few blood platelets)
  • Fatigue (from having too few red blood cells, changes in metabolism, or other factors)

Some of the most effective drugs against brain tumors tend to have fewer of these side effects than other common chemo drugs. Most side effects usually go away after treatment is finished. There are often ways to lessen these side effects. For example, drugs can often help prevent or reduce nausea and vomiting.

Some chemo drugs can also cause other, less common side effects. For example, cisplatin and carboplatin can also cause kidney damage and hearing loss. Your doctor will check your kidney function and hearing if you are getting these drugs. Some of these side effects might last after treatment is stopped.

Be sure to report any side effects to your medical team while getting chemo, so you can be treated promptly. Sometimes, the doses of the drugs may need to be reduced or treatment may need to be delayed or stopped to prevent the effects from getting worse.

Targeted drug therapy

Targeted drug treatments focus on specific abnormalities present within cancer cells. By blocking these abnormalities, targeted drug treatments can cause cancer cells to die. Targeted therapy drugs are available for certain types of brain tumors, and many more are being studied in clinical trials. Targeted drugs don’t yet play a large role in treating brain or spinal cord tumors, but some of them might be helpful for certain types of tumors. Your doctor may have your tumor cells tested to see whether targeted therapy is likely to be an effective treatment for your brain tumor.

Bevacizumab (Avastin, Mvasi, Zirabev)

Bevacizumab is a man-made version of an immune system protein called a monoclonal antibody. This antibody targets vascular endothelial growth factor (VEGF), a protein that helps tumors form new blood vessels (a process known as angiogenesis), which they need in order to grow.

This drug is used mainly to treat some types of gliomas (especially fast-growing ones such as glioblastomas) that come back after initial treatment. It might also be useful in treating recurrent meningiomas.

When used alone or added to chemotherapy, this drug can help shrink some tumors or extend the time until they start growing again, although it does not seem to help people live longer. It can also help lower the dose of the steroid drug dexamethasone needed to help reduce swelling in the brain, which is especially important for patients sensitive to steroid side effects.

Bevacizumab is given by intravenous (IV) infusion, usually once every 2 weeks.

Common side effects include high blood pressure, tiredness, bleeding, low white blood cell counts, headaches, mouth sores, loss of appetite, and diarrhea. Less common but possibly serious side effects include blood clots, internal bleeding, heart problems, and holes (perforations) in the intestines. This drug can also slow wound healing, so usually it can’t be given within a few weeks of surgery.

Everolimus (Afinitor)

Everolimus works by blocking a cell protein known as mTOR, which normally helps cells grow and divide into new cells. For subependymal giant cell astrocytomas (SEGAs) that can’t be removed completely by surgery, This drug may shrink the tumor or slow its growth for some time, although it’s not clear if it can help people with these tumors live longer.

Everolimus is a pill taken once a day. Common side effects include mouth sores, increased risk of infections, nausea, loss of appetite, diarrhea, skin rash, feeling tired or weak, fluid buildup (usually in the legs), and increases in blood sugar and cholesterol levels. A less common but serious side effect is damage to the lungs, which can cause shortness of breath or other problems.

Alternating electric field therapy

Researchers have found that exposing some types of tumor cells to alternating electric fields also known as tumor treating fields, can interfere with the cells’ ability to grow and spread. A wearable device known as Optune, which generates such electric fields, is now an option to help treat some people with glioblastomas. For this treatment, the head is shaved, and 4 sets of electrodes are placed on the scalp. The electrodes are attached to a battery pack (kept in a backpack) and are worn for most of the day. They generate mild electric currents that are thought to affect tumor cells in the brain more than normal cells.

Optune can be used:

  • Along with chemotherapy in people with newly diagnosed glioblastoma after treatment with surgery and radiation therapy. It may help people live longer than if they just get chemotherapy alone.
  • Instead of chemotherapy in people whose glioblastoma has come back after initial treatment. It hasn’t been shown to help people live longer than chemotherapy in this situation, but it tends to have much milder side effects.

Side effects of the device tend to be minor, and can include skin irritation at the electrode sites, trouble sleeping, mood changes, and a slightly increased risk of headaches and seizures.

Alternative medicine

Little research has been done on complementary and alternative brain tumor treatments. So far, no dietary supplements (including vitamins, minerals, and herbal products) have been shown to clearly help lower the risk of brain or spinal cord tumors progressing or coming back. No alternative treatments have been proved to cure brain tumors. This doesn’t mean that no supplements will help, but it’s important to know that none have been proven to do so. However, complementary treatments may help you cope with the stress of a brain tumor diagnosis.

Some complementary treatments that may help you cope include:

  • Acupuncture
  • Art therapy
  • Exercise
  • Meditation
  • Music therapy
  • Relaxation exercises

Talk with your doctor about your options.

Rehabilitation after treatment

Because brain tumors can develop in parts of the brain that control motor skills, speech, vision and thinking, rehabilitation may be a necessary part of recovery. Depending on your needs, your doctor may refer you to:

  • Physical therapy to help you regain lost motor skills or muscle strength
  • Occupational therapy to help you get back to your normal daily activities, including work, after a brain tumor or other illness
  • Speech therapy with specialists in speech difficulties (speech pathologists) to help if you have difficulty speaking
  • Tutoring for school-age children to help kids cope with changes in their memory and thinking after a brain tumor

Brain tumor prognosis

If you have a brain tumor, your prognosis (outlook) will depend on several factors, including:

  • your age
  • the type of brain tumor you have
  • where the tumor is in your brain
  • how effective the treatment is
  • your general health

Survival rates are difficult to predict because brain tumors are uncommon and there are many different types. Generally, around 15 out of every 100 people with a cancerous brain tumor will survive for 10 years or more after being diagnosed.

The numbers in the table below come from the Central Brain Tumor Registry of the United States (https://cbtrus.org) and are based on people who were treated between 2001 and 2015. As can be seen below, survival rates for some types of brain and spinal cord tumors can vary widely by age, with younger people tending to have better outlooks than older people. The survival rates for those 65 or older are generally lower than the rates for the ages listed below.

These numbers are for some of the more common types of brain and spinal cord tumors. Accurate numbers are not readily available for all types of tumors, often because they are rare or are hard to classify.

Survival rates can give you an idea of what percentage of people with the same type of brain or spinal cord tumor are still alive a certain amount of time (such as 5 years) after they were diagnosed. They can’t tell you how long you will live, but they may help give you a better understanding of how likely it is that your treatment will be successful.

Keep in mind that survival rates are estimates and are often based on previous outcomes of large numbers of people who had a specific type of tumor, but they can’t predict what will happen in any particular person’s case. These statistics can be confusing and may lead you to have more questions. Your doctor is familiar with your situation; ask how these numbers may apply to you.

A relative survival rate compares people with the same type of tumor to people in the overall population. For example, if the 5-year relative survival rate for a specific type of brain tumor is 35.1%, it means that people who have that tumor are, on average, about 35.1% as likely as people who don’t have that tumor to live for at least 5 years after being diagnosed.

Figure 6. Brain tumor 5-year relative survival

brain tumor 5-year relative survival

Footnotes: Localized = confined to primary site; Regional = spread to regional lymph nodes; Distant = cancer has metastasized; Unknown = unstaged

[Source 3 ]

Table 1. Survival rates for common adult brain and spinal cord tumors

Type of Brain Tumor5-Year Relative Survival Rate
Age
20-4445-5455-64
Low-grade (diffuse) astrocytoma73%46%26%
Anaplastic astrocytoma58%29%15%
Glioblastoma22%9%6%
Oligodendroglioma90%82%69%
Anaplastic oligodendroglioma76%67%45%
Ependymoma/anaplastic ependymoma92%90%87%
Meningioma84%79%74%

Footnotes:

  • These numbers don’t take everything into account. Survival rates are grouped here based on tumor type and a person’s age. But other factors, such as the location of the tumor, whether it can be removed (or destroyed) completely, and if the tumor cells have certain gene or chromosome changes, can also affect your outlook.
  • People now being diagnosed with brain or spinal cord tumors may have a better outlook than these numbers show. Treatments improve over time, and these numbers are based on people who were diagnosed and treated at least five years earlier.
  • Remember, these survival rates can’t predict what will happen to any individual person. If you find these statistics are confusing and you have more questions, talk to your doctor to better understand your specific situation.
[Source 6 ]

Can I lower my risk of the tumor progressing or coming back?

If you have (or had) a brain or spinal cord tumor, you probably want to know if there are things you can do to reduce your risk of the tumor progressing or coming back, such as exercising, eating a certain type of diet, or taking nutritional supplements. At this time, not enough is known about brain and spinal cord tumors to say for sure if there are things you can do that will help.

Adopting healthy behaviors such as not smoking, eating well, getting regular physical activity, and staying at a healthy weight might help, but no one knows for sure. However, scientists do know that these types of changes can have positive effects on your health that can extend beyond your risk of brain tumors or other cancers.

Living as a brain or spinal cord tumor survivor

Talk with your doctor about developing your survivorship care plan. This plan might include:

  • A suggested schedule for follow-up exams and tests
  • A schedule for other tests you might need in the future, such as tests to look for long-term health effects from your tumor or its treatment, or early detection (screening) tests for other types of cancer
  • A list of possible late- or long-term side effects from your treatment, including what to watch for and when you should contact your doctor
  • Diet and physical activity suggestions

You might have side effects from the tumor itself or from its treatment, which can range from very mild to fairly severe. Some side effects might last a long time or might not even show up until years after you have finished treatment. Your doctor visits are a good time to ask questions and talk about any changes or problems you notice or concerns you have.

Once you have recovered from treatment, your doctors will try to determine if damage was done to the brain or other areas. Careful physical exams and imaging tests (CT or MRI scans) might be done to determine the extent and location of any long-term changes in the brain.

Many types of doctors and other health professionals might help look for these changes and help you recover. For example:

  • A neurologist (a doctor who specializes in medical treatment of the nervous system) may assess your physical coordination, muscle strength, and other aspects of nervous system function.
  • If you have muscle weakness, paralysis, or numbness, you will likely be seen by physical and/or occupational therapists and perhaps a physiatrist (a doctor who specializes in rehabilitation) while in the hospital and/or as an outpatient for physical therapy.
  • If your speech is affected, a speech therapist (speech-language pathologist) will help improve your communication skills.
  • If needed, an ophthalmologist (a doctor who specializes in eye problems) will check your vision, and an audiologist may check your hearing.
  • After surgery, you may also see a psychiatrist or psychologist to determine the extent of any changes caused by the tumor or surgery. If you get radiation therapy and/or chemotherapy, this process may be repeated again after treatment is finished.
  • If you were treated with surgery or radiation therapy for a tumor near the base of the brain, pituitary hormone production may be affected. You might need to see an endocrinologist (a doctor who specializes in hormone disorders). If hormone levels are affected, you might need hormone treatments to restore normal levels for the rest of your life.

If the tumor comes back

If the tumor does recur, your treatment options will depend on the type and location of the tumor, what treatments you’ve had before, and your current health and preferences.

References
  1. Louis, D.N., Perry, A., Reifenberger, G. et al. The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. Acta Neuropathol 131, 803–820 (2016). https://doi.org/10.1007/s00401-016-1545-1
  2. Key Statistics for Brain and Spinal Cord Tumors. https://www.cancer.org/cancer/brain-spinal-cord-tumors-adults/about/key-statistics.html
  3. Cancer Stat Facts: Brain and Other Nervous System Cancer. https://seer.cancer.gov/statfacts/html/brain.html
  4. Sung H, Ferlay J, Siegel RL, Laversanne M, Soerjomataram I, Jemal A, Bray F. Global Cancer Statistics 2020: GLOBOCAN Estimates of Incidence and Mortality Worldwide for 36 Cancers in 185 Countries. CA Cancer J Clin. 2021 May;71(3):209-249. doi: 10.3322/caac.21660
  5. National Center for Biotechnology Information, U.S. National Library of Medicine. Hypothalamus. https://www.ncbi.nlm.nih.gov/pubmedhealth/PMHT0022790/
  6. Survival Rates for Selected Adult Brain and Spinal Cord Tumors. https://www.cancer.org/cancer/brain-spinal-cord-tumors-adults/detection-diagnosis-staging/survival-rates.html
Health Jade Team 3

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