Brain & Spinal Cord Tumors & Treatment

What are adult brain and spinal cord tumors?
Brain and spinal cord tumors are masses of abnormal cells that have grown out of control. In most other parts of the body, it is very important to distinguish between benign (non-cancerous) and malignant (cancerous) tumors. Benign tumors in other parts of the body do not grow into nearby tissues or spread to distant areas, so they are almost never life threatening. One of the main reasons cancers are so dangerous is because they can spread throughout the body.

Although brain tumors rarely spread to other parts of the body, most of them can spread through the brain tissue. Even so-called benign tumors can, as they grow, destroy and compress normal brain tissue, causing damage that is often disabling and sometimes fatal. For this reason, doctors usually speak of “brain tumors” rather than “brain cancers.” The main concerns with brain and spinal cord tumors are how readily they spread through the rest of the brain or spinal cord and whether they can be removed and not come back.

Brain and spinal cord tumors are different in adults and children. They often form in different areas, develop from different cell types, and may have a different outlook and treatment.

Parts of Brain

The main areas of the brain include the cerebrum, cerebellum, and brain stem. Each of these parts has a special function.

Cerebrum 

The cerebrum is the large, outer part of the brain. It has 2 hemispheres (halves) and controls 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.

The symptoms caused by a tumor in a cerebral hemisphere depend on where the tumor starts. Common symptoms include:

• Seizures
• Trouble speaking
• A change of mood such as depression
• A change in personality
• Weakness or paralysis of part of the body
• Changes in vision, hearing, or other sensations

Basal ganglia 

The basal ganglia are structures deeper within the brain that help control our muscle movements. Tumors or other problems in this part of the brain typically cause weakness, but in rare cases can cause tremor or other involuntary movements.

Cerebellum

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 fine movements of arms and legs, problems swallowing or synchronizing eye movements, and changes in rhythm of speech.

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 (which carry signals directly between the brain and the face, eyes, tongue, mouth, and some other areas) start in the brain stem.

Tumors in this critical area of the brain may cause weakness, stiff muscles, or problems with sensation, eye movement, hearing, facial movement, 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 may not be possible to surgically remove tumors in this area.

Spinal cord 

The spinal cord, like the brain stem, contains bundles of very long nerve fibers that carry signals controlling muscles, sensation or feeling, and bladder and bowel control. Spinal cord tumors may cause weakness, paralysis, or numbness. Because the spinal cord is such a narrow structure, tumors within it usually cause symptoms on both sides of the body (for example, weakness or numbness of both legs). This is different from brain tumors, which usually 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). Tumors of the spinal cord in the neck can cause symptoms in the arms and legs, as well as affect bowel and bladder function. Spinal cord tumors below the neck may only affect the legs and bowel and bladder function.

Cranial nerves 

The cranial nerves extend directly out of the base of the brain (as opposed to coming out of the spinal cord). Tumors starting in cranial nerves may cause vision problems, trouble swallowing, hearing loss in one or both ears, or facial paralysis, numbness, or pain.

Types of brain and spinal cord tumors 

Tumors that start in the brain (primary brain tumors) are not the same as tumors that start in other organs, such as the lung or breast, and then spread to the brain (metastatic or secondary brain tumors). In adults, metastatic tumors to the brain are actually more common than primary brain tumors. These tumors are not treated the same way. For example, breast or lung cancers that spread to the brain are treated differently from tumors that start in the brain.

Primary brain tumors can start in almost any type of tissue or cell in the brain or spinal cord. Some tumors have a mixture of cell types. Tumors in different areas of the central nervous system may be treated differently and have a different prognosis (outlook).

Gliomas

Gliomas are not a specific type of brain tumor. Glioma is a general term for a group of tumors that start in glial cells. A number of tumors can be considered gliomas, including glioblastoma (also known as glioblastoma multiforme), astrocytomas, oligodendrogliomas, and ependymomas. About 3 out of 10 of all brain tumors are gliomas. Most fast-growing brain tumors are gliomas.

Astrocytomas

Astrocytomas are tumors that start in glial cells called astrocytes. About 2 out of 10 brain tumors are astrocytomas.

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

Astrocytomas are often classified as low grade, intermediate grade, or high grade, based on how the cells look under the microscope.

• Low-grade astrocytomas tend to be slow growing.
• Intermediate-grade astrocytomas, or anaplastic astrocytomas, grow at a moderate rate.
• The highest-grade astrocytoma, known as glioblastoma (or glioblastoma multiforme), is the fastest growing. These tumors make up about two-thirds of astrocytomas and are the most common malignant brain tumors in adults.

Oligodendrogliomas

These tumors start in brain cells called oligodendrocytes. Like astrocytomas, most of these can grow into (infiltrate) nearby brain tissue and cannot be completely removed by surgery. Oligodendrogliomas sometimes spread along the CSF pathways but rarely spread outside the brain or spinal cord. Very aggressive forms of these tumors are known as anaplastic oligodendrogliomas. Only about 2% of brain tumors are oligodendrogliomas.

Ependymomas

These tumors arise from ependymal cells, which line the ventricles. They can range from fairly low-grade (less aggressive) tumors to higher grade ones, which are called anaplastic ependymomas. Only about 2% of brain tumors are ependymomas.

Unlike astrocytomas and oligodendrogliomas, ependymomas usually do not grow into (infiltrate) normal brain tissue. As a result, some (but not all) ependymomas can be completely removed 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 surgical cure, but treatment can cause side effects related to nerve damage.

Meningiomas

Meningiomas begin in the meninges, the layers of tissue that surround the outer part of the brain and spinal cord. Meningiomas account for about 1 out of 3 primary brain and spinal cord tumors. They are the most common brain tumors in adults (although strictly speaking, they are not actually brain tumors).

The risk of these tumors increases with age. They occur about twice as often in women. In some cases these tumors run in families, especially 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 I (benign) tumors have cells that look the most like normal cells. They make up about 80% of meningiomas. Most of these 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 II (atypical) meningiomas have cells that look slightly more abnormal. About 15% to 20% of meningiomas are grade II. They are more likely to come back (recur) after surgery.
• Grade III (anaplastic) meningiomas have cells that look the most abnormal. They make up only about 1% to 3% of meningiomas. They tend to grow quickly and are the most likely to come back after treatment. Some may even spread to other parts of the body.

Medulloblastomas

Medulloblastomas develop from neuroectodermal cells (primitive nerve cells) in the cerebellum. They are fast-growing tumors and often spread throughout the CSF pathways, but they can be treated by surgery, radiation therapy, and chemotherapy.

Medulloblastomas occur much more often in children than in adults. They are part of a class of tumors called primitive neuroectodermal tumors (PNETs) that can also start in other parts of the central nervous system. They are discussed in more detail in our document, Brain and Spinal Cord Tumors in Children.

Gangliogliomas

Gangliogliomas contain both neurons and glial cells. These tumors are very uncommon in adults and can usually be cured by surgery alone or surgery combined with radiation therapy.

Schwannomas (neurilemmomas)

Schwannomas develop from Schwann cells, which surround and insulate cranial nerves and other nerves. They make up about 9% of all CNS tumors.

Schwannomas are benign 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. They may 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.

Craniopharyngiomas

These slow-growing tumors start above the pituitary gland but below the brain itself. They 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.



Symptoms of Brain & Spinal Cord Tumors
Brain and spinal cord tumours may cause weakness or paralysis in parts of the body. Some people also have trouble balancing or have seizures.

Symptoms of brain tumours include:

• nausea and/or vomiting
• headaches
• drowsiness
• difficulty speaking or remembering words
• short-term memory problems
• disturbed vision, hearing, smell or taste
• loss of consciousness
• general irritability, depression or personality changes - this is sometimes only noticed by family or friends.

Symptoms of spinal cord tumours include:

• back and neck pain
• numbness or tingling in the arms or legs
• clumsiness or difficulty walking
• loss of bowel or bladder control (incontinence).

Causes of Brain & Spinal Cord Tumors
The causes of most brain and spinal cord tumours are unknown. However, there are a few known risk factors for malignant brain tumours:

Radiotherapy 
People who have had radiation to the head, usually to treat another type of cancer, may be at an increased risk of developing a tumour. This may affect people who had radiotherapy for childhood leukaemia.

Family history 
It is possible to have a genetic predisposition to developing a tumour. This means that you may have a fault in your genes, passed down from your parents, that increases your risk. For example, some people have a genetic condition called neurofibromatosis, which causes nerve tissue to grow tumours.

Mobile phones 
Some researchers have studied whether long-term or excessive use of mobile phones increases a person's risk of developing a brain tumour. It is possible that there may be an increased risk of developing a glioma in people with high levels of mobile phone use (i.e. more than 30 minutes a day). However, there is insufficient scientific evidence to link regular mobile phone use to brain tumours. Research is continuing in this area.

Treatment of Brain & Spinal Cord Tumors

Brain and spinal cord tumors can often be hard to treat and may require care from a team of several different doctors. This team is often led by a neurosurgeon, a doctor who uses surgery to treat brain and nervous system tumors. Several types of treatment may be used to treat brain and spinal cord tumors, including:

• Surgery
• Radiation therapy
• Chemotherapy
• Targeted therapy
• Other types of drugs

Surgery

For brain and spinal cord tumors, surgery may be done for different reasons:

• To get a biopsy sample to determine the type of tumor
• To remove as much of the tumor as possible
• To help prevent or treat 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 to remove the tumor

In most cases, the first step in brain 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 cure many tumors, including some low-grade astrocytomas, ependymomas, craniopharyngiomas, gangliogliomas, and meningiomas.

Tumors that tend to spread widely into nearby brain tissue such as anaplastic astrocytomas or glioblastomas cannot be cured by surgery. But surgery can 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 may not be a good option in some cases, such as if the tumor is deep within the brain, if it is in a part of the brain that can’t be removed, such as the brain stem, or if a person is unable to tolerate a major operation for other health reasons.

Craniotomy 

During a craniotomy, the surgeon makes an opening in the skull to reach the tumor. 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 may need to be shaved before surgery. The neurosurgeon first makes an incision in the scalp, and the skin is folded back. A special type of drill is used to remove the piece of the skull over the tumor.

The surgeon can remove the tumor in several ways depending on how hard or soft it is, and whether it has many or just a few blood vessels. One way is to cut it out with a scalpel or special scissors. Some tumors are soft and can be removed with simple suction devices. In other cases, a probe attached to an ultrasonic generator may be placed into the tumor to break it up and liquefy it. A small vacuum device is then used to suck it out.

In most cases the removed piece of bone is put back in place and fastened to the skull with metal screws and plates, wires, or special stitches. You may have 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. The drains are usually removed after a few days. Recovery time in the hospital is usually 4 to 6 days, although this could vary according to the size and location of the tumor and the patient's general health. Healing around the surgery site usually takes several weeks.

Surgery to place a shunt or ventricular access catheter

If a tumor blocks the flow of the CSF, it can increase pressure inside the skull. This can cause symptoms like headaches, nausea, and drowsiness, and may even be life-threatening.

Shunts may be temporary or permanent. They may be placed before or after the surgery to remove the tumor. Shunt placement is normally a straightforward procedure that takes about an hour. As with any operation, complications may develop, such as bleeding or infection. 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.

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 rays or small particles to kill cancer cells. This type of treatment is given by a doctor called a radiation oncologist. Radiation therapy may be used in different situations:

• After surgery to try to kill any remaining tumor cells
• As the main treatment if surgery is not a good option and medicines are not effective
• To help prevent or relieve symptoms
• Types of radiation therapy

In most cases, the radiation is focused precisely on the tumor from a source outside the body. This is called external beam radiation therapy (EBRT). This type of radiation therapy is much like getting an x-ray, but the dose of radiation is much higher.

Before your treatments start, the radiation team will measure carefully to determine the correct angles for aiming the radiation beams and the proper dose of radiation. In most cases, the total dose of radiation is divided into daily fractions (usually given Monday through Friday) over several weeks. At each 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. 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 high doses of radiation to the tumor with 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) 

IMRT is an advanced form of 3D therapy. It uses a computer-driven machine that actually 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.

Conformal proton beam radiation therapy 

Proton beam therapy is related to 3D-CRT and uses a similar approach. 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. Doctors can use this property to 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 meningiomas), but it is not clear if this approach will be useful with tumors that are infiltrative or mixed with normal brain tissue (such as astrocytomas or glioblastomas). There are only a handful of proton beam centers in the United States at this time.

Stereotactic radiosurgery/stereotactic radiotherapy 

This type of treatment delivers a large, precise radiation dose to the tumor area in a single session (radiosurgery) or in a few sessions (radiotherapy). (There is no actual surgery involved in this treatment.) 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's health is too poor for surgery.

First, a head frame is attached to the skull to help precisely aim the radiation beams. 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.

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.

Brachytherapy (internal radiotherapy)

 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 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, then 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.

Chemotherapy

Chemotherapy (chemo) uses anti-cancer drugs that are usually given into a vein (IV) or taken by mouth. These drugs enter the bloodstream and reach almost all areas of the body. However, many chemo drugs are not able to enter the brain and reach tumor cells.

For some brain tumors, the drugs may be given directly into the cerebrospinal fluid (CSF), 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 (see the “Surgery for adult brain and spinal cord tumors” section).

In general, chemotherapy is used for faster growing tumors. Some types of brain tumors, such as medulloblastoma and lymphoma, tend to respond better to chemotherapy.

Chemotherapy is most often used along with other types of treatment such as surgery and/or radiation therapy. Chemotherapy may 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 chemotherapy drugs that may be used to treat brain tumors include:

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

These drugs may be used alone or in various combinations, depending on the type of brain tumor. Doctors give chemo 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.

Other drug

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

Corticosteroids

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

Anti-seizure drugs (anti-epileptics)

Drugs may also be prescribed to lower the chance of seizures, which may happen in people with brain tumors. Different anti-seizure drugs may be used depending on a patient's circumstances. 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

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.

Lifestyle Changes after  
You can’t change the fact that you have had a brain or spinal cord tumor. What you can change is how you live the rest of your life – making choices to help you stay healthy and feel as well as you can. This can be a time to look at your life in new ways. Maybe you are thinking about how to improve your health over the long term. Some people even start during treatment.

Making healthier choices
For many people, a diagnosis of a brain or spinal cord tumor helps them focus on their health in ways they may not have thought much about in the past. Are there things you could do that might make you healthier? Maybe you could try to eat better or get more exercise. Maybe you could cut down on the alcohol, or give up tobacco. Even things like keeping your stress level under control may help. Now is a good time to think about making changes that can have positive effects for the rest of your life. You will feel better and you will also be healthier.

Eating better

If treatment caused weight changes or eating or taste problems, do the best you can and keep in mind that these problems usually get better over time. You might find it helps to eat small portions every 2 to 3 hours until you feel better. You may also want to ask your health care team about seeing a dietitian, an expert in nutrition who can give you ideas on how to deal with these treatment side effects.

One of the best things you can do after treatment is put healthy eating habits into place. You may be surprised at the long-term benefits of some simple changes, like increasing the variety of healthy foods you eat. Getting to and staying at a healthy weight, eating a healthy diet, and limiting your alcohol intake may lower your risk for a number of types of cancer, as well as having many other health benefits.

Rest, fatigue, and exercise

If you were sick and not very active during treatment, it is normal for your fitness, endurance, and muscle strength to decline. Any plan for physical activity should fit your own situation. An older person who has never exercised will not be able to take on the same amount of exercise as a 20-year-old who plays tennis twice a week. If you haven’t exercised in a few years, you will have to start slowly – maybe just by taking short walks.

If you are very tired, you will need to balance activity with rest. It is OK to rest when you need to. Sometimes it’s really hard for people to allow themselves to rest when they are used to working all day or taking care of a household, but this is not the time to push yourself too hard. Listen to your body and rest when you need to.

Keep in mind exercise can improve your physical and emotional health.

• It improves your cardiovascular (heart and circulation) fitness.
• Along with a good diet, it will help you get to and stay at a healthy weight.
• It makes your muscles stronger.
• It reduces fatigue and helps you have more energy.
• It can help lower anxiety and depression.
• It can make you feel happier.
• It helps you feel better about yourself.

And long term, we know that getting regular physical activity plays a role in helping lower the risk of some cancers, as well as having other health benefits.