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Brain Tumors

Brain Tumors in Children and Young Adults

Pediatric brain tumors are a group of diseases in which there is abnormal growth of tissues of the brain. This abnormal cell growth can be low grade(very slow growing cancer cells) or malignant (contains cancer cells with a faster growth rate). The brain controls memory, learning, movement, the senses (hearing, sight, smell, taste, touch) and emotion. Other than leukemia, brain tumors are the most common cancer that occurs in children and young adults.

This summary will refer to tumors that begin in the brain tissue (primary brain tumors). Sometimes cancer found in the brain has started in other body parts and has spread to the brain (metastasized). These tumors are not covered in this summary.

There are no specific signs or symptoms of a brain tumor. A doctor should be consulted if your child has morning headaches and vomiting, fatigue, declining school performance, visual problems, problems moving an arm or leg, problems feeling with hand(s) or a foot, seizures or behavioral changes. If there are symptoms concerning to the doctor, they may order a computed tomographic (CT scan), a special x-ray that uses a computer to create pictures of the brain. A magnetic resonance imaging (MRI) scan, which uses magnetic waves to make a picture of the brain, may also be done.

If tumors are detected, surgery is needed to determine what type of tumors they are. A neurosurgeon will take a small sample of tissue or remove the entire mass (if possible). Then the brain cells will be examined under the microscope. Removal of a small amount of tissue is called a biopsy. Sometimes a biopsy is done by making a small hole in the skull and extracting a small sample of tissue. The best thing you can do for your child is get to a neurosurgeon experienced in pediatric brain tumors. Find more information on our Pediatric and Young Adult Brain Tumor program.

There are many different types of childhood brain tumors. The chance of recovery (prognosis) and choice of treatment depend on the type of tumor, the location of the tumor in the brain, the extent of tumor spread, your child's age, and general state of health. So, one of the hardest parts can be waiting for the pathologist to determine exactly what kind of tumor it is. This can take days, but it most commonly takes a week, and sometimes two. Rest assured that most often, post-surgical treatment does not need to start right away, so your child can usually heal from surgery before therapy has to start.

Types of pediatric brain tumors

Once a pediatric brain tumor is diagnosed, additional tests are done to find out the type of tumor. Tumor cells obtained from a biopsy are carefully looked at under the microscope to see how different they look from normal cells. This tells the doctor the "grade" of the tumor. "High grade" tumor cells (the most malignant type) look quite different from normal brain cells. They grow faster and are more malignant than low grade tumor cells. To plan treatment, the doctor needs to know the type of tumor and the grade of tumor.

There is no staging for childhood brain tumors, although for most types a spinal MRI is needed. Rather, they are grouped according to their location in the brain, the appearance of the cells and the behavior of the cells. The following groups are used for childhood brain tumors:

Infratentorial tumors

50% of childhood brain tumors. These tumors occur in the lower portion of the brain and include:

  • Medulloblastoma (primitive neuroectodermal tumor) usually forms in the cerebellum (the lower back part of the brain). The cerebellum controls movement, balance and posture. About 20% of childhood brain tumors are medulloblastoma.
  • Cerebellar astrocytoma are tumors of brain cells called astrocytes. They begin in the cerebellum (the lower back part of the brain), the part of the brain that controls movement, balance and posture. About 15-25% of childhood brain tumors are cerebellar astrocytomas. The majority are termed "pilocytic" which are slow-growing and rarely spread to other areas of the brain. This is the lowest "grade" of brain tumor, and some doctors call them "benign" because they don't spread in the vast majority of cases. However, if just a single cell is left inside (which an MRI cannot see), this "benign" tumor can recur. For this reason, the term "low grade" is most commonly used.
  • Ependymoma is a tumor that begins in the cells that line (ependyma) the hollow spaces within the brain (the ventricles) which contain cerebrospinal fluid (CSF). Abnormal growth of the ependymal cells can block the circulation of the CSF through the brain and spinal cord. Tumor cells may spread via the CSF to other areas of the brain and/or spinal cord. Ten percent (10%) of childhood brain tumors are ependymomas.
  • Brain stem glioma is a tumor that begins in the brain stem, that portion of the brain connected to the spinal cord. The brain stem is the lowest part of the brain, located just above the back of the neck. The brain stem controls breathing, heart rate, nerves and muscles used in seeing, hearing, walking, talk and eating.
Supratentorial tumors

Supratentorial tumors are those tumors which occur in the upper portion of the brain and include:

  • Cerebral astrocytomas are tumors of brain cells called astrocytes. They begin in the area of the brain called the cerebrum (top of the brain). Astrocytomas can be low grade or high grade tumors.
  • Ependymoma is a tumor of the cells that line (ependyma) the hollow spaces within the brain (the ventricles) which contain the cerebrospinal fluid (CSF). Abnormal growth of the ependymal cells can block the circulation of the CSF through the brain and spinal cord. Supratentorial ependymoma begin in the upper part of the brain. Tumor cells may spread to other areas of the brain and/or spinal cord.
  • Craniopharyngioma is a nonmalignant tumor that generally occurs just above the pituitary gland, which is located at the bottom of the brain above the roof of the mouth. The pituitary gland (also called the master gland) secretes a number of hormones that help us control and respond to stress, regulates growth and sexual development, and regulates energy metabolism and the body's ability to fight infections. It is also involved in the control of the ability to concentrate urine. Craniopharyngiomas produce a variety of effects by affecting the pituitary glands ability to produce or secrete its hormones.
  • Germ cell tumors arise from cells which give rise to the sex organs, which are found in the brain during early development. There are several types of germ cells tumors which usually occur in the center of the brain. They can spread to other parts of the brain.
  • Primitive neuroectodermal tumors and pineal tumors are supratentorial tumors found in the upper part of the brain or in and around the pineal gland at the center of the brain. These tumors can spread to other parts of the brain.
  • Visual pathway and hypothalamic gliomas are tumors that begin in astrocytes and are usually low grade tumors of the optic nerve, the optic chiasm and the optic tract (the visual pathway). Visual pathway gliomas occur with increased incidence in patients with Neurofibromatosis Type I. In infants and children with hypothalamic glioma, the child often appears euphoric, but emaciated. Both visual pathway and hypothalamic gliomas show symptoms of visual impairment.

Recurrent brain tumor

Recurrent disease means the cancer (tumor) has come back (recurred) after it has been treated. It may recur in its original location, in another part of the central nervous system or throughout the body.

Treatment option overview

There are treatments for children with brain tumors. Three types of treatment are used:

  • Surgery (removing the tumor in an operation performed by a neurosurgeon).
  • Radiation therapy (high dose x-rays to kill tumor cells).
  • Chemotherapy (drugs that kill tumor cells).

More than one method of treatment is usually used to treat childhood brain tumors.

The best treatment plan for your child is made by experienced doctors working together.

Coordinating the plan is the pediatric oncologist, a doctor who specializes in childhood cancer. Also involved in treating a child with a brain tumor is the pediatric neurosurgeon, the pediatric neurologist, a radiation oncologist, a psychologist, child life specialists and other health care providers who specialize in the types of care children with brain tumors require.

Treatment options are based on the type of brain tumor and the location of the brain tumor. Complete (or near complete) removal of the brain tumor is often possible and usually the preferred option. If the tumor cannot be completely removed, radiation therapy and/or chemotherapy will usually be given. Some tumors, such as medulloblastoma, require chemotherapy and radiation even when the tumor is completely removed.

Radiation therapy

Radiation therapy uses high energy radiation from x-rays (and other sources such as gamma rays) to shrink tumors by killing cancer cells. Radiation therapy usually comes from a machine outside of the child's body (external radiation source). Clinical trials are evaluating different ways to give external radiation (e.g. hyperfractionated radiation, which gives several small doses of radiation daily) in certain childhood brain tumors. Clinical trials are also testing ways to decrease or delay radiation therapy for younger children, as radiation therapy does affect growth and brain development in younger children. If radiation is recommended, one of the best things you can do for your child is get an opinion from a radiation oncologist who works at a referral center. They will have equipment with the least side effects, such as IMRT (Intensity-Modulated Radiation Therapy). There is a list of questions for you to ask at the end of this section.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be given by mouth, injected into a vein (intravenous) or injected into a muscle (intramuscular). Chemotherapy is called a systemic treatment because the drug enters the blood steam, travels throughout the body and can kill cancer cells through the body. Clinical trials are studying whether chemotherapy can be used as a way to delay, modify or eliminate the use of radiation therapy in younger children with brain tumors. Other clinical trials are studying the use of chemotherapy prior to or during radiation therapy.

Biological therapy is also being studied in clinical trials as a method of treating childhood brain tumors. Biological therapy uses materials (substances) that are normally produced by the body and can now be produced in the laboratory, to boost, direct or restore the body's natural defenses against disease. It is also referred to as immunotherapy or biological response modifier therapy.

Treatment on clinical trials

Treatment for childhood brain tumors depends on the type of tumor, the grade of tumor, tumor location within the brain, your child's age and overall health of your child.

Your child may receive treatment that is considered standard therapy based on its effectiveness in patients in past clinical trials, or you may choose to have your child entered on a clinical trial. Clinical trials are designed to test new potentially better therapies against therapy considered as standard in hope of finding better ways to treat childhood brain tumors. Clinical trials are ongoing in most parts of the country for childhood brain tumors. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

Medulloblastoma

The therapy will depend on whether all of the tumor was removed surgically and the age of the child. There are several components of therapy:

  1. Surgery - complete removal of tumor if possible
  2. Radiation therapy
  3. Chemotherapy
  4. Cerebrospinal fluid diversion is a process sometimes needed to drain fluid from the brain in cases where fluid has accumulated. Drainage of fluid is accomplished using a shunt (a long thin tube) that is placed into a ventricle (fluid-filled spaces within the brain) and threaded under the skin to the abdomen. The fluid carried away from the brain by the shunt is absorbed in the abdominal cavity.

Treatment of "average risk" medulloblastoma includes:

  1. Surgery
  2. Radiation therapy to the brain and spinal cord with or without chemotherapy.

Treatment of "poor risk" medulloblastoma (some tumor remains after surgery, and there may be spread in the spinal fluid) includes:

  1. Surgery.
  2. Radiation therapy to the brain and spinal cord
  3. Chemotherapy

Treatment of medulloblastoma in children younger than 3 years of age will probably include:

  1. Surgery
  2. Chemotherapy
  3. Radiation therapy with or without chemotherapy
  4. A clinical trial using chemotherapy followed by peripheral blood stem cell rescue.

Treatment for recurrent medulloblastoma may include:

  1. Surgery with or without chemotherapy
  2. A clinical trial of high dose chemotherapy and stem cell transplantation (using bone marrow or peripheral blood stem cells) with radiation therapy
  3. A clinical trial using novel therapy.

Cerebellar astrocytomas

Initial treatment for cerebellar astrocytomas is usually surgery. If the tumor is completely removed, more treatment may not be needed. The child is closely observed, and undergoes regular MRI scans, watching for symptoms to appear or change as well as looking for changes on the MRI pictures. This observation is also known as watchful waiting.

If cancer cells remain after surgery, treatment depends upon the location of the remaining tumor and the age of the child. Treatment may include:

  • Watchful waiting
  • Surgery
  • Radiation therapy
  • Chemotherapy

Treatment of recurrent cerebellar astrocytoma may include:

  1. Surgery
  2. Radiation therapy
  3. Chemotherapy
  4. A clinical trial of new, novel, anticancer drugs

Infratentorial Ependymoma

Initial treatment is usually surgery to remove as much tumor as possible, followed by radiation therapy to the tumor site.

If tumor cells remain after surgery and have spread within the brain or spine, radiation therapy to the whole brain and spine is likely.

The children younger than 3 years of age, chemotherapy may be given to delay, modify or eliminate the radiation therapy. Clinical trials for ependymoma are underway to better define the usefulness of radiation and chemotherapy.

For recurrent ependymomas, the following treatments may be used:

  • Surgery
  • Radiation therapy
  • Chemotherapy
  • A clinical trial of new therapies

Supratentorial Ependymoma

Treatment is usually surgery, followed by radiation therapy and chemotherapy. Clinical trials are evaluating surgery, followed by chemotherapy with or without radiation therapy. Clinical trials are evaluating radiation therapy with or without chemotherapy.

In children younger than 3 years of age, chemotherapy may be given to delay, modify or eliminate the radiation therapy.

For recurrent ependymomas, the following treatments may be used:

  • Surgery
  • Radiation therapy
  • Chemotherapy
  • A clinical trial of new therapies

Brain stem glioma

Treatment of diffuse intrinsic brain stem glioma may include:

  • Radiation therapy
  • Radiation therapy combined with chemotherapy
  • A clinical trial of novel chemotherapy

When the tumor is entirely within the brainstem, most commonly a biopsy is not done because it would harm the child. This is especially true when the tumor is located in the pons. If the tumor is in the medulla and pons and there is a portion that extends out of the brainstem, the doctors may feel that the child would benefit from this part of the tumor being removed.

Treatment of focal or low-grade gliomas may include:

  • Surgery
  • Cerebrospinal fluid diversion followed by watchful waiting.
  • A clinical trial of surgery followed by watchful waiting.

Treatment of brain stem glioma in children with neurofibromatosis, Type I is usually watchful waiting. These tumors are usually slow growing and may not need treatment for many years.

Treatment of recurrent brain stem glioma depends on the type of tumor, where in the brain it recurs and previously received treatment.

Treatment of recurrent diffuse intrinsic brain stem glioma may include:

  • Surgery
  • Palliative care to reduce relieve symptoms and improve quality of life
  • A clinical trial of new, novel therapies

Treatment of recurrent focal or low grade childhood brain stem glioma may include:

  • Surgery
  • Chemotherapy

Cerebral astrocytoma/malignant glioma

Low grade cerebral astrocytoma treatment depends upon whether all the cancer can be removed with surgery. If all the tumor can be removed, no additional treatment may be needed.

If there is cancer remaining after surgery, radiation therapy may be given or radiation therapy can be delayed until the remaining tumor started to grow again.

Chemotherapy is being studied in some groups of children, including those younger than 5 years of age.

High grade cerebral astrocytoma is usually treated with surgery, followed by radiation therapy and chemotherapy. Children younger than 3 years of age may be given chemotherapy to delay radiation therapy or allow a lower dose of radiation to be given.

Clinical trials are evaluating surgery followed by chemotherapy with or without radiation therapy.

Treatment for recurrent cerebral astrocytoma depends upon tumor type, where in the brain the tumor recurs and previous treatments. Surgery and chemotherapy may be given for recurrent low grade cerebral astrocytoma. Surgery may be used for recurrent high grade cerebral astrocytoma. You may want to consider entering a clinical trial of new chemotherapy agents or new ways of giving chemotherapy.

Craniopharyngioma

Treatment is usually surgery with or without radiation therapy. Chemotherapy has no defined role in this tumor. Clinical trials involving new, novel therapies may be ongoing.

Central Nervous System Germ Cell Tumor

Treatment is radiation therapy with or without chemotherapy, depending on the tumor cell type.

Visual pathway glioma

Treatment may include:

  1. Watchful waiting
  2. Surgery
  3. Radiation therapy
  4. Chemotherapy
  5. Clinical trials evaluating chemotherapy followed by radiation therapy

Treatment of recurrent visual pathway glioma may include:

  1. Surgery
  2. Radiation therapy
  3. Chemotherapy
  4. Clinical trials of new, novel chemotherapy agents

Supratentorial primitive neuroectodermal tumor

Treatment usually consists of surgery to remove as much of the tumor as possible, followed by craniospinal radiation therapy with or without chemotherapy. Clinical trials are evaluating combined modality treatment, as well as new chemotherapy agents.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, the staff of the Pediatric and Young Brain Tumor Program are leaders at the national level (Children's Oncology Group, sponsored by the NCI) in the development of treatment for children with brain tumors. We specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Our pediatric oncologists are all subspecialty board certified, and represent decades of experience. Our physicians are national leaders in the development of treatments for children with cancer. Our Pediatric Intensive Care Unit is staffed around-the-clock by in-hospital pediatric intensive care specialists, all board-certified in Pediatric Critical Care Medicine. As a result, mortality rates in the Children's Hospital PICU are much lower than the national average.

The Radiation Oncology Department works closely with our team, providing state-of-the-art therapy. Cutting edge radiation tools, such as 3-D conformal radiation therapy ( which includes intensity-modulated radiation therapy (IMRT) and 3-D radiation treatments) , precisely deliver radiation to chest area lymphomas while minimizing dose to normal tissues. We were one of the first centers to offer IMRT for pediatric patients.

We have taken on the prevention of infection as a quality and safety program; the implementation of preventive antibiotics at key therapy timepoints is constantly monitored. In addition, our referring physicians are our partners in the care of each child, from the very beginning. Because they also care for the brothers and sisters of our patients, we enlist them as teammates in getting preventive immunizations in our patient's family members.

State-of-the-art cancer therapy is technically complex, and we meet that demand with high-tech, safe care. Yet, in this environment of high-tech supportive medicine, our families find a gentle, comprehensive, informative, and comfortable medical home. Art therapists and child life specialists are found among a medical staff that embraces each family completely. Our school re-entry program helps each individual child find their way back to school and other important parts of their life, during therapy.

Because early diagnosis and treatment are critical to good outcomes and to parents peace of mind, rapid referrals are a priority. Children and young adults referred to the Department of Pediatric Hematology/Oncology can be seen quickly, either on the same day or the next day.

Consider also the value of a second opinion from a pediatric hematologist/oncologist at Cleveland Clinic Children's. A second opinion can provide reassurance that a prescribed treatment is best, or it may offer families fresh options. For out-of-town patients or those too ill to travel, the e-Cleveland Clinic second opinion service offers convenient online access to pediatric specialists. Find out more by calling 800.223.2273, ext. 4-3223, or by visiting our Web site at www.eclevelandclinic.org.

We offer you the words of the parents we work with:

Everyone seems to work as a team, with the focus being on the children and their families. Again thanks to you and your staff. They portray the excellence that is missing in healthcare today. We feel so blessed that we brought our son to Cleveland Clinic.

Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Children's Cancer Answer line at 216.444.5517 or 800.223.2273, extension 4-5517, select option 1.

We wish you and your child well.

For other websites regarding childhood cancer:

Source: National Institutes of Health, National Cancer Institute, Children's Oncology Group

This information is provided by the Cleveland Clinic Health System and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition.