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Cancer Medicine :: Central Nervous System - Childhood

Central Nervous System - Childhood

Treatment Options

In general, tumors in children are uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That's why more than 60% of children are treated as part of a clinical trial. Clinical trials are research studies that compare standard treatments (the best proven treatments available) with newer treatments that may be more effective. Investigating new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

          

To take advantage of these newer treatments, all children with a CNS tumor should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children and have access to the latest research. A doctor who specializes in treating children with a tumor is called a pediatric oncologist. In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, nutritionists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Treatment overview

Descriptions of the most common treatment options for a CNS tumor are listed below. Treatment options and recommendations depend on several factors, including the type of CNS tumor, whether it is cancerous, the stage and/or grade of the tumor, possible side effects, the family's preferences, and the child's age and overall health. Radiation therapy is often limited in children younger than three years old, because extensive radiation therapy can cause learning and memory problems later on in the child's life.

Type of CNS Tumor Treatment Commonly Used
Astrocytoma

Low-grade astrocytoma: Surgery alone, or surgery plus radiation therapy, or radiation therapy alone for older children; surgery plus chemotherapy or limited radiation therapy for younger children

High-grade astrocytoma: Surgery plus radiation therapy and chemotherapy

Brain stem glioma Radiation therapy, with or without chemotherapy
Ependymoma Surgery and radiation therapy; possibly chemotherapy
Germ cell tumor Surgery and radiation therapy, with or without chemotherapy
Medulloblastoma Surgery, radiation therapy, and chemotherapy

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. It is almost always used to determine the type of tumor, either through a biopsy or during treatment when the tumor can be removed without significant damage to the brain. A neurosurgeon is a doctor who specializes in treating a tumor in the brain or spine with surgery. Sometimes, a tumor spreads and grows between normal nerve cells, making surgery difficult. In certain cases, surgery can damage parts of the brain near the tumor, affecting arm and leg movement, breathing, swallowing, eye movement, or consciousness. Some tumors cannot be removed by surgery because of their location; these tumors are called inoperable; in these instances, the doctor will recommend other treatment options. 

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Stereotactic radiosurgery is a way to deliver high doses of radiation therapy directly to a tumor and not to healthy tissue. This technique makes detailed, three-dimensional maps of the brain and tumor, so doctors can pinpoint where to direct the radiation treatment. It works best for certain noncancerous tumors and a tumor that is only in one part of the brain. 

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, headaches, and loose bowel movements. Most side effects go away soon after treatment is finished. However, in the long term, radiation therapy can sometimes interfere with the growth and development of the child's brain. Therefore, the doctor may choose to treat the tumor in another way.

Chemotherapy

Chemotherapy is the use of drugs to kill tumor cells, usually by stopping the tumor cells' ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating a tumor with medication. A chemotherapy regimen usually consists of a specific number of cycles given over a set period time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Chemotherapy is effective for many cancerous types of brain tumors. Depending on the tumor type, chemotherapy may be given immediately after a biopsy or surgery or after radiation therapy. In some instances, chemotherapy is used at the same time as radiation therapy.

Chemotherapy may also be given directly into the spinal canal, in a procedure called intrathecal chemotherapy, to treat tumor cells on the surface of the brain and spine. This procedure is still being researched in clinical trials and may not be available everywhere.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

The medications used to treat a tumor are continually being evaluated. Talking with your child's doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. 

Palliative/supportive care

A CNS tumor and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the disease, an important part of care is relieving a child's symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the child with his or her physical, emotional, and social needs.

Palliative care can help a child at any stage of illness. Children often receive treatment for the tumor and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and families report that they are more satisfied with treatment.

Before treatment begins, talk with your child's health care team about the possible side effects of your child's specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if your child is experiencing a problem, so it is addressed as quickly as possible. 

Recurrent CNS tumor

A remission is when the tumor cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the tumor will come back. While many remissions are permanent, it's important to talk with the doctor about the possibility of the tumor returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the tumor does return. 

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your child's doctor will talk about the treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but may be used in a different combination or given at a different pace. Your child's doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent tumor.

For a recurrent CNS tumor, the next phase of treatment depends on three factors:

The type of tumor

Whether the tumor recurred where it originally began or in another part of the brain or body

The type of treatment the child received for the original tumor

Depending on individual circumstances, the doctor may recommend surgery, radiation therapy, chemotherapy, and/or stem cell/bone marrow transplantation.

A recurrent tumor may bring up emotions such as disbelief or fear. You and your family are encouraged to talk with the health care team about these feelings and ask about support services to help you cope. 

If treatment fails

Although treatment is successful for the majority of children with a tumor, sometimes it is not. If a child's tumor cannot be cured or controlled, this is called an advanced or terminal tumor. This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child's doctor and health care team to express your family's feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child's health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. 

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving.

Coping with Side Effects

Fear of treatment side effects is common after a diagnosis of a CNS tumor, but it may help to know that preventing and controlling side effects is a major focus of your child's health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for a CNS tumor are described in detail within the Treatment section. 

Before treatment begins, talk with your child's doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends often play an important role in the care of a child with a CNS tumor. 

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. 

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. 

After Treatment

After treatment for a CNS tumor ends, talk with your child's doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child's recovery for the coming months and years. All children treated for a CNS tumor should have life-long, follow-up care.

Based on the type of treatment the child received, the doctor will determine what examinations and tests are needed to check for long-term side effects and the possibility of secondary tumors. For example, craniospinal (head and spine) radiation therapy can cause cognitive (thought-process) and endocrine (hormonal) symptoms over time, although the severity can vary greatly depending on the dose given and the age of the child. Similarly, the risks and potential side effects of surgery vary widely, depending on the location of the tumor and how it grew. Likewise, the risks of chemotherapy and the likelihood of secondary tumors also strongly depend on the drugs and doses used. Your child's doctor can recommend the necessary screening tests. Follow-up care should also address the child's quality of life, including any developmental or emotional concerns. 

The child's family is encouraged to organize and keep a record of the child's medical information. That way, as the child enters adulthood, he or she has a clear, written history of the diagnosis, the treatment given, and the doctor's recommendations about the schedule for follow-up care. The doctor's office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. 

Children who have had a CNS tumor can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child's needs.

Latest Research

Doctors are working to learn more about CNS tumors, ways to prevent them, how to best treat them, and how to provide the best care to children diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child's doctor about the diagnostic and treatment options best for your child.

New medications. Doctors are examining new types of drugs that may better control tumor growth.

Improved imaging techniques. Imaging techniques are being developed and refined that help surgeons pinpoint the tumor's location, to reduce or prevent tissue damage to the healthy parts of the brain during treatment.

Functional MRI (fMRI) is an imaging technique that identifies the parts of the brain that control speech, hearing, vision, touch, and movement. The specific locations of these functions are slightly different in every person, so fMRI allows surgeons to plan surgery around these areas.

Image-guided stereotaxis allows surgeons to visualize and operate on the brain using three-dimensional outlines of the brain and the tumor. Along with specialized software, these images help guide the surgeon to the tumor. Many tumors that were once considered inoperable can now be removed with this technique.

Magnetic resonance spectroscopy (MRS) and positron emission tomography (PET) scans are also being used to examine tumor metabolic activity. Whether these techniques will improve a doctor's ability to predict how a tumor will grow and spread is still being researched.

Improved methods of delivering radiation treatment. Doctors are experimenting with new techniques for delivering radiation therapy to certain types of tumors. These methods make detailed, three-dimensional maps of the brain and tumor, so doctors can focus the radiation on the tumor and avoid damage to the nearby healthy tissue. This can reduce a child's exposure to radiation and lessen the long-term side effects.

Targeted therapy. Recent research has found specific genes, proteins, and other factors unique to different types of CNS tumors. Treatments aimed at these factors, called targeted therapy, are now being studied. Targeted therapy is a treatment that targets the cancer's specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells.

Immunotherapy. Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Researchers are studying how well these drugs work and how safe they are for children with CNS tumors.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current CNS tumor treatments in order to improve patients' comfort and quality of life.

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