What Is a Brain Tumor?

A brain tumor is an abnormal growth of tissue in the brain or central spine that can disrupt proper brain function. Doctors refer to a tumor based on where the tumor cells originated, and whether they are cancerous (malignant) or not (benign).

• Benign: The least aggressive type of brain tumor is often called a benign brain tumor. They originate from cells within or surrounding the brain, do not contain cancer cells, grow slowly, and typically have clear borders that do not spread into other tissue.
• Malignant: Malignant brain tumors contain cancer cells and often do not have clear borders. They are considered to be life threatening because they grow rapidly and invade surrounding brain tissue.
• Primary: Tumors that start in cells of the brain are called primary brain tumors. Primary brain tumors may spread to other parts of the brain or to the spine, but rarely to other organs.
• Metastatic: Metastatic or secondary brain tumors begin in another part of the body and then spread to the brain. These tumors are more common than primary brain tumors and are named by the location in which they begin.

There are over 120 types of brain and central nervous system 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.

A brain tumor is a mass or growth of abnormal cells in your brain or close to your brain.

Many different types of brain tumors exist. Some brain tumors are noncancerous (benign), and some brain tumors are cancerous (malignant). Brain tumors can begin in your brain (primary brain tumors), or cancer can begin in other parts of your body and spread to your brain (secondary, or metastatic, brain tumors).

How quickly a brain tumor grows can vary greatly. The growth rate as well as location of a brain tumor determines how it will affect the function of your nervous system.

Brain tumor treatment options depend on the type of brain tumor you have, as well as its size and location.

What are the parts of the brain?

The brain is a soft, spongy mass of tissue. It is protected by:

• The bones of the skull
• Three thin layers of tissue(meninges)
• Watery fluid(cerebrospinal fluid) that flows through spaces between the meninges and through spaces (ventricles) within the brain.

The brain directs the things we choose to do (like walking and talking) and the things our body does without thinking (like breathing). The brain is also in charge of our senses (sight, hearing, touch, taste, and smell), memory, emotions, and personality.

A network of nerves carries messages back and forth between the brain and the rest of the body. Some nerves go directly from the brain to the eyes, ears, and other parts of the head. Other nerves run through the spinal cord to connect the brain with the other parts of the body.

Within the brain and spinal cord, glial cells surround nerve cells and hold them in place.

The three major parts of the brain control different activities:

• Cerebrum:The cerebrum uses information from our senses to tell us what is going on around us and tells our body how to respond. It controls reading, thinking, learning, speech, and emotions.

The cerebrum is divided into the left and right cerebral hemispheres. The right hemisphere controls the muscles on the left side of the body. The left hemisphere controls the muscles on the right side of the body.

• Cerebellum: The cerebellum controls balance for walking and standing, and other complex actions.
• Brain stem:The brain stem connects the brain with the spinal cord. It controls breathing, body temperature, blood pressure, and other basic body functions.

Types of primary brain tumors

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There are many types of primary brain tumors. Primary brain tumors are named according to the type of cells or the part of the brain in which they begin. For example, most primary brain tumors begin in glial cells. This type of tumor is called a glioma.

Among adults, the most common types are:

• Astrocytoma: The tumor arises from star-shaped glial cells called astrocytes. It can be any grade. In adults, an astrocytoma most often arises in the cerebrum.
• Grade I or II astrocytoma: It may be called a low-grade glioma.
• Grade III astrocytoma: It’s sometimes called a high-grade or an anaplastic astrocytoma.
• Grade IV astrocytoma: It may be called a glioblastomaor malignant astrocytic glioma.
• Meningioma: The tumor arises in the meninges. It can be grade I, II, or III. It’s usually benign (grade I) and grows slowly.
• Oligodendroglioma: The tumor arises from cells that make the fatty substance that covers and protects nerves. It usually occurs in the cerebrum. It’s most common in middle-aged adults. It can be grade II or III.

Among children, the most common types are:

• Medulloblastoma: The tumor usually arises in the cerebellum. It’s sometimes called a primitive neuroectodermal tumor. It is grade IV.
• Grade I or II astrocytoma: In children, this lowgrade tumor occurs anywhere in the brain. The most common astrocytoma among children is juvenile pilocytic astrocytoma. It’s grade I.
• Ependymoma: The tumor arises from cells that line the ventricles or the central canal of the spinal cord. It’s most commonly found in children and young adults. It can be grade I, II, or III.
• Brain stem glioma: The tumor occurs in the lowest part of the brain. It can be a low-grade or high-grade tumor. The most common type is diffuse intrinsic pontine glioma.

Brain tumor grades

Doctors group brain tumors by grade. The grade of a tumor refers to the way the cells look under a microscope:

• Grade I: The tissue is benign. The cells look nearly like normal brain cells, and they grow slowly.
• Grade II: The tissue is malignant. The cells look less like normal cells than do the cells in a Grade I tumor.
• Grade III: The malignant tissue has cells that look very different from normal cells. The abnormal cells are actively growing (anaplastic).
• Grade IV: The malignant tissue has cells that look most abnormal and tend to grow quickly.

Cells from low-grade tumors (grades I and II) look more normal and generally grow more slowly than cells from high-grade tumors (grades III and IV).

Over time, a low-grade tumor may become a high-grade tumor. However, the change to a high-grade tumor happens more often among adults than children.

Types

• Choroid plexus carcinoma
• Craniopharyngioma
• Embryonal tumors
• Ependymoma
• Medulloblastoma
• Pineoblastoma

Choroid plexus carcinoma

A choroid plexus carcinoma is a rare cancerous (malignant) brain tumor that occurs mainly in children.

A choroid plexus carcinoma begins near the brain tissue that secretes cerebrospinal fluid. A noncancerous tumor of this area is called a choroid plexus papilloma. As the tumor grows, it can affect the function of nearby structures in the brain, causing excess fluid in the brain (hydrocephalus), irritability, nausea or vomiting, and headaches.

Treatment and chance of recovery (prognosis) depend on the tumor’s size, location, whether it has spread, and your child’s age and general health.

Diagnosis

A choroid plexus carcinoma most often occurs in children under 2 years old. Tests and procedures used to diagnose choroid plexus carcinoma include:

• Physical exam.Diagnosing a choroid plexus carcinoma usually starts with a medical history review and a neurological exam. During this procedure, your child’s vision, hearing, balance, coordination and reflexes are tested.
• Brain imaging tests.Tests to create images of your brain may include magnetic resonance imaging (MRI) and computerized tomography (CT). An MRI also helps your doctor to plan the surgery.
• Genetic tests.Tests to identify genetic aspects of some choroid plexus carcinomas are available. Ask your doctor about genetic counseling.

Treatment in children usually differs from treatment in adults. If your child receives a diagnosis of choroid plexus carcinoma, ask your doctor to refer you to a specialist in treating children with brain tumors (pediatric neuro-oncologist, pediatric radiation oncologist).

Treatment

Treatment of a choroid plexus carcinoma is often surgery followed by chemotherapy, Radiation Therapy or both.

• The goal of surgery is to remove the entire tumor, when possible. But because delicate and important structures may be nearby, doctors sometimes can’t remove the entire tumor. Generally, people who undergo surgery for a choroid plexus carcinoma will need additional therapy after surgery.
• After surgery, about half of the people who had excess fluid on the brain (hydrocephalus) experience relief from this symptom. Others may need to have a temporary drain (shunt) placed at the surgical site.
• Chemotherapy may be used in addition to surgery and radiation therapy to help control the tumor.
• Radiation therapy.Radiation may be used after surgery, even if the entire tumor was successfully removed. Radiation may also be used later if the tumor grows back.

Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill tumor cells. Advanced technologies such as stereotactic radiosurgery, proton therapy, intensity-modulated radiation therapy (IMRT) and intraoperative radiation therapy (IORT) help treat the cancer effectively while sparing healthy tissues, such as the eyes, optic nerves, brain, brainstem and spinal cord.

• Clinical trials.Clinical trials are studies of new treatments or new ways of using existing treatments.

Craniopharyngioma

Pituitary gland and hypothalamus

Craniopharyngioma is a rare type of noncancerous (benign) brain tumor.

Craniopharyngioma begins near the brain’s pituitary gland, which secretes hormones that control many body functions. As a craniopharyngioma slowly grows, it can affect the function of the pituitary gland and other nearby structures in the brain.

Craniopharyngioma can occur at any age, but it occurs most often in children and older adults. Symptoms include gradual changes in vision, fatigue, excessive urination and headaches. Children with craniopharyngioma may grow slowly and may be smaller than expected.

Diagnosis

Tests and procedures used to diagnose craniopharyngioma include:

• Physical exam.Diagnosing a craniopharyngioma usually starts with a medical history review and a neurological exam by your doctor. During this procedure, your vision, hearing, balance, coordination, reflexes, and growth and development are tested.
• Blood tests.Blood tests may reveal changes in hormone levels that indicate a tumor is affecting your pituitary gland.
• Imaging tests.Tests to create images of your brain may include X-rays, magnetic resonance imaging (MRI) and computerized tomography (CT).

Treatment

Craniopharyngioma treatment options include:

• Surgery to remove all or most of the tumor is most often recommended for people with craniopharyngioma. What type of operation is performed depends on the location and size of your tumor.

Open craniopharyngioma surgery (craniotomy) involves opening the skull to gain access to the tumor. During minimally invasive craniopharyngioma surgery (transsphenoidal procedure), special surgical tools are inserted through your nose. The tools pass through a natural corridor to the tumor, without affecting the brain.

When possible, surgeons remove the entire tumor. But because there are often many delicate and important structures nearby, doctors sometimes don’t remove the entire tumor in order to ensure a good quality of life after the operation. In those situations, other treatments may be used after surgery.

• Radiation therapy.External beam radiation therapy may be used after surgery to treat craniopharyngioma. This treatment uses powerful energy beams, such as X-rays and protons, to kill tumor cells. During external beam radiation therapy, you lie on a table while a machine precisely aims the energy to the tumor cells.

Specialized external beam radiation technology, such as proton beam therapy and intensity-modulated radiation therapy (IMRT), allows doctors to carefully shape and aim the radiation beam so that it delivers treatment to the tumor cells and spares nearby healthy tissue.

A type of radiation therapy called stereotactic radiosurgery may be recommended in uncommon situations when the tumor doesn’t touch the bundle of nerve fibers that transmits visual information from your eye to your brain (optic nerve). Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells.

Another type of radiation therapy called brachytherapy involves placement of radioactive material directly into the tumor where it can radiate the tumor from the inside.

• Chemotherapy is a drug treatment that uses chemicals to kill tumor cells. Chemotherapy can be injected directly into the tumor so that the treatment reaches the target cells and isn’t likely to damage nearby healthy tissue.
• Treatment for papillary craniopharyngioma.An uncommon type of craniopharyngioma called papillary craniopharyngioma may respond to targeted therapy. Targeted therapy is a drug treatment that focuses on specific abnormalities within the tumor cells that allows them to survive.
• Nearly all papillary craniopharyngioma cells contain a mutation in a gene called the BRAF gene. Targeted therapy aimed at this mutation may be a treatment option. Specialized laboratory testing can reveal whether your craniopharyngioma contains papillary cells and whether those cells have the BRAF gene mutation.
• Clinical trials.Clinical trials are studies of new treatments or new ways of using existing treatments. A clinical trial gives you the chance to try the latest treatments, but the side effects may not be known. Ask your doctor whether you’re eligible to participate in clinical trials.

Embryonal tumors

Embryonal tumors of the central nervous system are cancerous (malignant) tumors that start in the fetal (embryonic) cells in the brain. Embryonal tumors can occur at any age, but most often occur in babies and young children.

Types of embryonal tumors include:

• The most common type of embryonal tumor, these fast-growing cancerous brain tumors start in the lower back part of the brain, called the cerebellum. The cerebellum is involved in muscle coordination, balance and movement. Medulloblastomas tend to spread through cerebrospinal fluid (CSF) to other areas around the brain and spinal cord, though they rarely spread to other areas of the body.
• Embryonal tumors with multilayered rosettes (ETMRs).Rare tumors that are cancerous, ETMRs typically occur in infants and young children. These aggressive tumors most often start in the largest part of the brain, called the cerebrum, which controls thinking and voluntary movement. ETMRs can also occur in other parts of the brain and are often characterized by a certain genetic change.
• These rare, fast-growing cancerous tumors typically occur in the brain or spinal cord of infants and young children.
• Atypical teratoid/rhabdoid tumors.These rare, cancerous tumors are most often found in the cerebellum of infants and children under 3 years of age.
• Other embryonal tumors.A category called embryonal tumors not otherwise specified includes most cancerous tumors that were formerly called primitive neuroectodermal tumors (PNETs), occurring in the brain and spinal cord.

Signs and symptoms of embryonal tumors vary, depending on the type of tumor, location, severity and other factors, such as pressure buildup within the brain. Symptoms may include, for example, headaches, nausea, vomiting, unusual tiredness, dizziness, double vision, unsteady walk, seizures or other issues.

Diagnosis

The process of diagnosis usually starts with a medical history review and a discussion of signs and symptoms. Tests and procedures used to diagnose embryonal tumors include those below, but additional tests may be needed to find out if the cancer has spread.

• Neurological exam.During this procedure, vision, hearing, balance, coordination and reflexes are tested. This helps determine which part of the brain might be affected by the tumor.
• Imaging tests.Imaging tests can help determine the location and size of the brain tumor. These tests are also very important to identify pressure or blockage of the CSF pathways. A computerized tomography (CT) scan or magnetic resonance imaging (MRI) may be done right away. These tests are often used to diagnose brain tumors. Advanced techniques, such as perfusion MRI and magnetic resonance spectroscopy, also may be used.
• Removal of tissue for testing (biopsy).A biopsy is usually not done before the tumor is surgically removed, but it may be recommended if the imaging features aren’t typical of embryonal tumors. The sample of suspicious tissue is analyzed in a lab to determine the types of cells.
• Removal of cerebrospinal fluid for testing (lumbar puncture).Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out cerebrospinal fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities. This test is only done after managing the pressure in the brain or removing the tumor.

Treatment

Treatment for embryonal tumors depends on the patient’s age (typically babies and young children), tumor type and location, tumor grade and extent, and other factors. Options include:

• Surgery to relieve fluid buildup in the brain.Some embryonal tumors may grow to block the flow of cerebrospinal fluid, which can cause a buildup of fluid that puts pressure on the brain (hydrocephalus). ). Surgery to create a pathway for the fluid to flow out of the brain (external ventricular drain or ventriculoperitoneal shunt) may be recommended. Sometimes this procedure can be combined with surgery to remove the tumor.
• Surgery to remove the tumor.A pediatric brain surgeon (neurosurgeon) removes as much of the tumor as possible, taking care not to harm nearby tissue. Typically, all children with embryonal tumors should receive additional treatments after surgery to target any remaining cells.
• Radiation therapy.A pediatric radiation oncologist administers radiation therapy to the brain and spinal cord using high-energy beams, such as X-rays or protons, to kill cancer cells. Standard radiation therapy can be used, but proton beam therapy — available at a limited number of major health care centers in the United States — delivers higher targeted doses of radiation to brain tumors, minimizing radiation exposure to nearby healthy tissue.
• Chemotherapy uses drugs to kill tumor cells. Typically, children with embryonal tumors receive these drugs as an injection into the vein (intravenous chemotherapy). Chemotherapy may be recommended after surgery or radiation therapy, or in certain cases, at the same time as radiation therapy. In some cases, high dose chemotherapy followed by stem cell rescue (a stem cell transplant using the patient’s own stem cells) may be used.
• Clinical trials.Clinical trials enroll eligible participants to study the effectiveness of new treatments or to study new ways of using existing treatments, such as different combinations or timing of radiation therapy and chemotherapy. These studies provide a chance to try the latest treatment options, though the risk of side effects may not be known. Talk with your doctor for advice.

To ensure correct diagnosis and treatment, children with embryonal tumors need to be seen at a center that has a team of pediatric specialists with expertise and experience in pediatric brain tumors, with access to the latest technology and treatments for children.

Ependymoma

Ependymoma is a type of tumor that can form in the brain or spinal cord. Ependymoma begins in the ependymal cells in the brain and spinal cord that line the passageways where the fluid (cerebrospinal fluid) that nourishes your brain flows.

Ependymoma can occur at any age, but most often occurs in young children. Children with ependymoma may experience headaches and seizures. Ependymoma that occurs in adults is more likely to form in the spinal cord and may cause weakness in the part of the body controlled by the nerves that are affected by the tumor.

Surgery is the primary treatment for ependymoma. For more aggressive tumors or for tumors that can’t be removed completely with surgery, additional treatments, such as radiation therapy or chemotherapy, may be recommended.

Diagnosis

Tests and procedures used to diagnose ependymoma include:

• Neurological exam.During a neurological exam, your doctor will ask you about your child’s signs and symptoms. He or she may check your child’s vision, hearing, balance, coordination, strength and reflexes. Problems in one or more of these areas may provide clues about the part of your child’s brain that could be affected by a brain tumor.
• Imaging tests.Imaging tests can help doctors determine the location and size of the brain tumor. MRI is often used to diagnose brain tumors, and it may be used along with specialized MRI imaging, such as magnetic resonance angiography. Because ependymoma can occur in both the brain and spine, imaging tests should be used to create pictures of both areas when a diagnosis of ependymoma is suspected.
• Removing cerebrospinal fluid for testing (lumbar puncture).Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities.

Based on your child’s test results, the doctor may suspect ependymoma and recommend surgery to remove the tumor. Once removed, the tumor cells will be tested in a laboratory to confirm the diagnosis. Specialized tests are used to determine the types of cells and their level of aggressiveness, which the doctor may use to guide treatment decisions.

Treatment

Ependymoma treatment options include:

• Surgery to remove the ependymoma.Brain surgeons (neurosurgeons) work to remove as much of the ependymoma as possible. The goal is to remove the entire tumor, but sometimes the ependymoma is located near sensitive brain or spinal tissue that makes that too risky.

If the entire tumor is removed during surgery, your child may not require any additional treatment. If some tumor remains, the neurosurgeon may recommend another operation to try to remove the rest of the tumor. Additional treatments, such as radiation therapy, may be recommended for more aggressive tumors or if all of the tumor can’t be removed.

• Radiation therapy.Radiation therapy uses high-energy beams, such as X-rays or protons, to kill cancer cells. During radiation therapy, your child lies on a table while a machine moves around him or her, directing beams to precise points in the brain.

Radiation therapy may be recommended after surgery to help prevent more aggressive tumors from recurring or if neurosurgeons weren’t able to remove the tumor completely.

Specialized techniques can help to ensure that treatment delivers radiation to the tumor cells and spares as much of the surrounding healthy tissue as possible. Conformal radiation therapy, intensity-modulated radiation therapy and proton therapy are types of radiation therapy that allow doctors to carefully and precisely deliver radiation.

• Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells. Radiosurgery is sometimes used when an ependymoma recurs after surgery and radiation.
• Chemotherapy uses drugs to kill cancer cells. Chemotherapy isn’t very effective for most cases of ependymoma. The role of chemotherapy is still largely experimental and is reserved for certain situations, such as when the tumor grows back despite surgery and radiation.
• Clinical trials.Clinical trials are studies of new treatments. These studies give you a chance to try the latest treatment options, but the risk of side effects may not be known. Ask your doctor whether your child might be eligible to participate in a clinical trial.

Medulloblastoma

Medulloblastoma (muh-dul-o-blas-TOE-muh) is a cancerous (malignant) brain tumor that starts in the lower back part of the brain, called the cerebellum. The cerebellum is involved in muscle coordination, balance and movement.

Medulloblastoma tends to spread through cerebrospinal fluid (CSF) — the fluid that surrounds and protects your brain and spinal cord — to other areas around the brain and spinal cord. This tumor rarely spreads to other areas of the body.

Medulloblastoma is a type of embryonal tumor — a tumor that starts in the fetal (embryonic) cells in the brain. Based on different types of gene mutations, there are at least four subtypes of medulloblastoma. Though medulloblastoma is not inherited, syndromes such as Gorlin’s syndrome or Turcot’s syndrome might increase the risk of medulloblastoma.

Signs and symptoms of medulloblastoma may include headaches, nausea, vomiting, tiredness, dizziness, double vision, poor coordination, unsteady walk and other concerns. These symptoms may be related to the tumor itself or be due to the buildup of pressure within the brain.

Medulloblastoma can occur at any age, but most often occurs in young children. Though medulloblastoma is rare, it’s the most common cancerous brain tumor in children. Children need to be seen at a center that has a team of pediatric specialists with expertise and experience in pediatric brain tumors, with access to the latest technology and treatments for children.

Diagnosis

The process of diagnosis usually starts with a medical history review and a discussion of signs and symptoms. Tests and procedures used to diagnose medulloblastoma include:

• Neurological exam.During this procedure, vision, hearing, balance, coordination and reflexes are tested. This helps determine which part of the brain might be affected by the tumor.
• Imaging tests.Imaging tests can help determine the location and size of the brain tumor. These tests are also very important to identify pressure or blockage of the CSF pathways. A computerized tomography (CT) scan or magnetic resonance imaging (MRI) may be done right away. These tests are often used to diagnose brain tumors. Advanced techniques, such as perfusion MRI and magnetic resonance spectroscopy, also may be used.
• Tissue sample testing (biopsy).A biopsy is usually not done, but it may be recommended if the imaging tests are not typical of medulloblastoma. The sample of suspicious tissue is analyzed in a lab to determine the types of cells.
• Removal of cerebrospinal fluid for testing (lumbar puncture).Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out cerebrospinal fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities. This test is only done after managing the pressure in the brain or removing the tumor.

Treatment

Treatment for medulloblastoma usually includes surgery followed by radiation or chemotherapy, or both. Age and general health, tumor subtype and location, tumor grade and extent, and other factors play a role in treatment decisions. Options include:

• Surgery to relieve fluid buildup in the brain.A medulloblastoma may grow to block the flow of cerebrospinal fluid, which can cause a buildup of fluid that puts pressure on the brain (hydrocephalus). Surgery to create a pathway for the fluid to flow out of the brain (external ventricular drain or ventriculoperitoneal shunt) may be recommended. Sometimes this procedure can be combined with surgery to remove the tumor.
• Surgery to remove the medulloblastoma.A pediatric or adult brain surgeon (neurosurgeon) removes the tumor, taking care not to harm nearby tissue. But sometimes it’s not possible to remove the tumor entirely because medulloblastoma forms near critical structures deep within the brain. All patients with medulloblastoma should receive additional treatments after surgery to target any remaining cells.
• Radiation therapy.A pediatric or adult radiation oncologist administers radiation therapy to the brain and spinal cord using high-energy beams, such as X-rays or protons, to kill cancer cells. Standard radiation therapy can be used, but proton beam therapy — available at a limited number of major health care centers in the United States — delivers higher targeted doses of radiation to brain tumors, minimizing radiation exposure to nearby healthy tissue.
• Chemotherapy uses drugs to kill tumor cells. Typically, children and adults with medulloblastoma receive these drugs as an injection into the vein (intravenous chemotherapy). Chemotherapy may be recommended after surgery or radiation therapy, or in certain cases, at the same time as radiation therapy. In some cases, high dose chemotherapy followed by stem cell rescue (a stem cell transplant using the patient’s own stem cells) may be used.
• Clinical trials.Clinical trials enroll eligible participants to study the effectiveness of new treatments or to study new ways of using existing treatments, such as different combinations or timing of radiation therapy and chemotherapy. These studies provide a chance to try the latest treatment options, though the risk of side effects may not be known. Talk with your doctor for advice.

Pineoblastoma

Pineoblastoma is a rare, aggressive type of cancer that begins in the cells of the brain’s pineal gland. Your pineal gland, located in the center of your brain, produces a hormone (melatonin) that plays a role in your natural sleep-wake cycle.

Pineoblastoma can occur at any age, but it tends to occur most often in young children. Pineoblastoma may cause headaches, sleepiness and subtle changes in the way the eyes move.

Pineoblastoma can be very difficult to treat. It can spread within the brain and the fluid (cerebrospinal fluid) around the brain, but it rarely spreads beyond the central nervous system. Treatment usually involves surgery to remove as much of the cancer as possible. Additional treatments may also be recommended.

Diagnosis

Tests and procedures used to diagnose pineoblastoma include:

• Imaging tests.Imaging tests can help your doctor determine the location and size of your child’s brain tumor. Magnetic resonance imaging (MRI) is often used to diagnose brain tumors, and advanced techniques, such as perfusion MRI and magnetic resonance spectroscopy, may also be used.

Additional tests might include computerized tomography (CT) and positron emission tomography (PET).

• Removing a sample of tissue for testing (biopsy).A biopsy can be done with a needle before surgery or during surgery to remove the pineoblastoma. The sample of suspicious tissue is analyzed in a laboratory to determine the types of cells and their level of aggressiveness.
• Removing cerebrospinal fluid for testing (lumbar puncture).Also called a spinal tap, this procedure involves inserting a needle between two bones in the lower spine to draw out cerebrospinal fluid from around the spinal cord. The fluid is tested to look for tumor cells or other abnormalities. In certain situations, cerebrospinal fluid may instead be collected during a biopsy procedure to remove suspicious tissue from the brain.

Treatment

Pineoblastoma treatment options include:

• Surgery to relieve fluid buildup in the brain.A pineoblastoma may grow to block the flow of cerebrospinal fluid, which can cause a buildup of fluid that puts pressure on the brain (hydrocephalus). An operation to create a way for the fluid to flow out of the brain may be recommended. Sometimes this procedure can be combined with a biopsy or surgery to remove the tumor.
• Surgery to remove the pineoblastoma.The brain surgeon (neurosurgeon) will work to remove the pineoblastoma with the goal of removing as much of the tumor as possible. But it’s often impossible to remove the tumor entirely because pineoblastoma forms near critical structures deep within the brain. Most children with pineoblastoma receive additional treatments after surgery to target the remaining cells.
• Radiation therapy.Radiation therapy uses high-energy beams, such as X-rays or protons, to kill cancer cells. During radiation therapy, your child lies on a table while a machine moves around him or her, directing beams to the brain and spinal cord, with additional radiation to the tumor. Because there is a high risk the tumor cells can spread beyond the initial site to other areas of the central nervous system, radiation therapy directed to the entire brain and spinal cord is recommended for children older than 3.
• Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be recommended after surgery or radiation therapy in children with pineoblastoma. In some cases, it’s used at the same time as radiation therapy. For larger tumors, chemotherapy may be used before surgery to shrink the tumor and make it easier to remove.
• Technically a type of radiation and not an operation, stereotactic radiosurgery focuses multiple beams of radiation on precise points to kill the tumor cells. Radiosurgery is sometimes used to treat pineoblastoma that recurs.
• Clinical trials.Clinical trials are studies of new treatments. These studies give you a chance to try the latest treatment options, but the risk of side effects may not be known. Ask your doctor whether your child might be eligible to participate in a clinical trial.

Symptoms

The signs and symptoms of a brain tumor vary greatly and depend on the brain tumor’s size, location and rate of growth.

General signs and symptoms caused by brain tumors may include:

• New onset or change in pattern of headaches
• Headaches that gradually become more frequent and more severe
• Unexplained nausea or vomiting
• Vision problems, such as blurred vision, double vision or loss of peripheral vision
• Gradual loss of sensation or movement in an arm or a leg
• Difficulty with balance
• Speech difficulties
• Confusion in everyday matters
• Personality or behavior changes
• Seizures, especially in someone who doesn’t have a history of seizures
• Hearing problems

Causes

Malignant glioma cells

Acoustic neuroma (schwannoma)

Brain tumors that begin in the brain

Primary brain tumors originate in the brain itself or in tissues close to it, such as in the brain-covering membranes (meninges), cranial nerves, pituitary gland or pineal gland.

Primary brain tumors begin when normal cells acquire errors (mutations) in their DNA. These mutations allow cells to grow and divide at increased rates and to continue living when healthy cells would die. The result is a mass of abnormal cells, which forms a tumor.

Primary brain tumors are much less common than are secondary brain tumors, in which cancer begins elsewhere and spreads to the brain.

Many different types of primary brain tumors exist. Each gets its name from the type of cells involved. Examples include:

• These tumors begin in the brain or spinal cord and include astrocytomas, ependymoma, glioblastomas, oligoastrocytomas and oligodendrogliomas.
• A meningioma is a tumor that arises from the membranes that surround your brain and spinal cord (meninges). Most meningiomas are noncancerous.
• Acoustic neuromas (schwannomas).These are benign tumors that develop on the nerves that control balance and hearing leading from your inner ear to your brain.
• Pituitary adenomas.These are mostly benign tumors that develop in the pituitary gland at the base of the brain. These tumors can affect the pituitary hormones with effects throughout the body.
• These are the most common cancerous brain tumors in children. A medulloblastoma starts in the lower back part of the brain and tends to spread through the spinal fluid. These tumors are less common in adults, but they do occur.
• Primitive neuroectodermal tumors (PNETs) are rare, cancerous tumors that start in embryonic (fetal) cells in the brain. They can occur anywhere in the brain.
• Germ cell tumors.Germ cell tumors may develop during childhood where the testicles or ovaries will form. But sometimes germ cell tumors move to other parts of the body, such as the brain.
• These rare, noncancerous tumors start near the brain’s pituitary gland, which secretes hormones that control many body functions. As the craniopharyngioma slowly grows, it can affect the pituitary gland and other structures near the brain.

Cancer that begins elsewhere and spreads to the brain

Secondary (metastatic) brain tumors are tumors that result from cancer that starts elsewhere in your body and then spreads (metastasizes) to your brain.

Secondary brain tumors most often occur in people who have a history of cancer. But in rare cases, a metastatic brain tumor may be the first sign of cancer that began elsewhere in your body.

Secondary brain tumors are far more common than are primary brain tumors.

Any cancer can spread to the brain, but the most common types include:

• Breast cancer
• Colon cancer
• Kidney cancer
• Lung cancer
• Melanoma

Risk factors

In most people with primary brain tumors, the cause of the tumor is not clear. But doctors have identified some factors that may increase your risk of a brain tumor. Risk factors include:

• Your age.Your risk of a brain tumor increases as you age. Brain tumors are most common in older adults. However, a brain tumor can occur at any age. And certain types of brain tumors occur almost exclusively in children.
• Exposure to radiation.People who have been exposed to a type of radiation called ionizing radiation have an increased risk of brain tumor. Examples of ionizing radiation include radiation therapy used to treat cancer and radiation exposure caused by atomic bombs.
• More common forms of radiation, such as electromagnetic fields from power lines and radiofrequency radiation from cellphones and microwave ovens, have not been proved to be linked to brain tumors.
• Family history of brain tumors.A small portion of brain tumors occur in people with a family history of brain tumors or a family history of genetic syndromes that increase the risk of brain tumors.

How are brain tumors diagnosed?

If you have symptoms that suggest a brain tumor, your doctor will give you a physical exam and ask about your personal and family health history. You may have one or more of the following tests:

• Neurologic exam: Your doctor checks your vision, hearing, alertness, muscle strength, coordination, and reflexes. Your doctor also examines your eyes to look for swelling caused by a tumor pressing on the nerve that connects the eye and the brain.
• MRI: A large machine with a strong magnet linked to a computer is used to make detailed pictures of areas inside your head. Sometimes a special dye (contrast material) is injected into a blood vessel in your arm or hand to help show differences in the tissues of the brain. The pictures can show abnormal areas, such as a tumor.
• CT scan: An x-ray machine linked to a computer takes a series of detailed pictures of your head. You may receive contrast material by injection into a blood vessel in your arm or hand. The contrast material makes abnormal areas easier to see. Your doctor may ask for other tests:
• Angiogram: Dye injected into the bloodstream makes blood vessels in the brain show up on an x-ray. If a tumor is present, the x-ray may show the tumor or blood vessels that are feeding into the tumor.
• Spinal tap: Your doctor may remove a sample of cerebrospinal fluid (the fluid that fills the spaces in and around the brain and spinal cord). This procedure is performed with local anesthesia. The doctor uses a long, thin needle to remove fluid from the lower part of the spinal column. A spinal tap takes about 30 minutes. You must lie flat for several hours afterward to keep from getting a headache. A laboratory checks the fluid for cancer cells or other signs of problems.
• Biopsy: The removal of tissue to look for tumor cells is called a biopsy. A pathologist looks at the cells under a microscope to check for abnormal cells. A biopsy can show cancer, tissue changes that may lead to cancer, and other conditions. A biopsy is the only sure way to diagnose a brain tumor, learn what grade it is, and plan treatment. Surgeons can obtain tissue to look for tumor cells in two ways:
• • Biopsy at the same time as treatment: The surgeon takes a tissue sample when you have surgery to remove part or all of the tumor. See the Surgery section.
  • Stereotactic biopsy: You may get local or general anesthesia and wear a rigid head frame for this procedure. The surgeon makes a small incision in the scalp and drills a small hole (a burr hole) into the skull. CT or MRI is used to guide the needle through the burr hole to the location of the tumor. The surgeon withdraws a sample of tissue with the needle. A needle biopsy may be used when a tumor is deep inside the brain or in a part of the brain that can’t be operated on.

However, if the tumor is in the brain stem or certain other areas, the surgeon may not be able to remove tissue from the tumor without harming normal brain tissue. In this case, the doctor uses MRI, CT, or other imaging tests to learn as much as possible about the brain tumor.

A person who needs a biopsy may want to ask the doctor the following questions:

• Why do I need a biopsy? How will the biopsy results affect my treatment plan?
• What kind of biopsy will I have?
• How long will it take? Will I be awake? Will it hurt?
• What are the chances of infection or bleeding after the biopsy? Are there any other risks?
• How soon will I know the results?
• If I do have a brain tumor, who will talk with me about treatment? When?

Radiation therapy for brain tumors

Radiation therapy kills brain tumor cells with high-energy x-rays, gamma rays, or protons.

Radiation therapy usually follows surgery. The radiation kills tumor cells that may remain in the area. Sometimes, people who can’t have surgery have radiation therapy instead.

Doctors use external and internal types of radiation therapy to treat brain tumors:

• External radiation therapy: You’ll go to a hospital or clinic for treatment. A large machine outside the body is aimed to direct beams of radiation at either the whole brain or more commonly, at specific portions of the brain. Some people need radiation aimed at the spinal cord also. The treatment schedule depends on your age, and the type and size of the tumor. Fractionated external beam therapy, in which small doses or fractions of radiation are given usually once each day, is the most common method of radiation therapy used for people with brain tumors. Giving the total dose of radiation over several weeks helps to protect healthy tissue in the area of the tumor. Treatments are usually 5 days a week for several weeks. A typical visit lasts less than an hour, and each treatment takes only a few minutes.Some treatment centers are studying other ways of delivering external beam radiation therapy:
  • Intensity-modulated radiation therapy or 3-dimensional conformal radiation therapy: These types of treatment use computers to more closely target the brain tumor to lessen the damage to healthy tissue.
  • Proton beam radiation therapy: The source of radiation is protons rather than X-rays. The doctor aims the proton beam at the tumor. The dose of radiation to normal tissue from a proton beam is less than the dose from an X-ray beam.
  • Stereotactic radiation therapy: Narrow beams of X-rays or gamma rays are directed at the tumor from different angles. For this procedure, you wear a rigid head frame. The therapy may be given during a single visit (stereotactic radiosurgery) or over several visits.
• Internal radiation therapy (implant radiation therapy or brachytherapy): Internal radiation isn’t commonly used for treating brain tumors and is under study. The radiation comes from radioactive material usually contained in very small implants called seeds. The seeds are placed inside the brain and give off radiation for months. They don’t need to be removed once the radiation is gone.

Some people have no or few side effects after treatment. Rarely, people may have nausea for several hours after external radiation therapy. The health care team can suggest ways to help you cope with this problem. Radiation therapy also may cause you to become very tired with each radiation treatment. Resting is important, but doctors usually advise people to try to stay as active as they can.

Also, external radiation therapy commonly causes hair loss from the part of the head that was treated. Hair usually grows back within a few months. Radiation therapy also may make the skin on the scalp and ears red, dry, and tender. The health care team can suggest ways to relieve these problems.

Sometimes radiation therapy causes brain tissue to swell. You may get a headache or feel pressure. The health care team watches for signs of this problem. They can provide medicine to reduce the discomfort. Radiation sometimes kills healthy brain tissue. Although rare, this side effect can cause headaches, seizures, or even death.

Radiation may harm the pituitary gland and other areas of the brain. For children, this damage could cause learning problems or slow down growth and development. In addition, radiation increases the risk of secondary tumors later in life.

You may want to ask your doctor these questions about radiation therapy:

• Why do I need this treatment?
• When will the treatments begin? When will they end?
• How will I feel during therapy? Are there side effects?
• What can I do to take care of myself during therapy?
• How will we know if the radiation is working?
• Will I be able to continue my normal activities during treatment?

Chemotherapy for brain tumors

Chemotherapy, the use of drugs to kill cancer cells, is sometimes used to treat brain tumors. Drugs may be given in the following ways:

• By mouth or vein (intravenous): Chemotherapy may be given during and after radiation therapy. The drugs enter the bloodstream and travel throu