Ependymoblastoma, Childhood

Treatment and Diagnosis:

If a person has symptoms that suggest a brain tumor, the doctor may perform one or more of the following procedures:

• Physical exam — The doctor checks general signs of health.
• Neurologic exam — The doctor checks for alertness, muscle strength, coordination, reflexes, and response to pain. The doctor also examines the eyes to look for swelling caused by a tumor pressing on the nerve that connects the eye and brain.
• CT scan — An x-ray machine linked to a computer takes a series of detailed pictures of the head. The patient may receive an injection of a special dye so the brain shows up clearly in the pictures. The pictures can show tumors in the brain.
• MRI — A powerful magnet linked to a computer makes detailed pictures of areas inside the body. These pictures are viewed on a monitor and can also be printed. Sometimes a special dye is injected to help show differences in the tissues of the brain. The pictures can show a tumor or other problem in the brain.

The doctor may ask for other tests:

• Angiogram — Dye injected into the bloodstream flows into the blood vessels in the brain to make them show up on an x-ray. If a tumor is present, the doctor may be able to see it on the x-ray.
• Skull x-ray — Some types of brain tumors cause calcium deposits in the brain or changes in the bones of the skull. With an x-ray, the doctor can check for these changes.
• Spinal tap — The 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 spinal column. A spinal tap takes about 30 minutes. The patient 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.
• Myelogram — This is an x-ray of the spine. A spinal tap is performed to inject a special dye into the cerebrospinal fluid. The patient is tilted to allow the dye to mix with the fluid. This test helps the doctor detect a tumor in the spinal cord.
• 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.

Surgeons can obtain tissue to look for tumor cells in three ways:

• Needle biopsy — The surgeon makes a small incision in the scalp and drills a small hole into the skull. This is called a burr hole. The doctor passes a needle through the burr hole and removes a sample of tissue from the brain tumor.
• Stereotactic biopsy — An imaging device, such as CT or MRI, guides the needle through the burr hole to the location of the tumor. The surgeon withdraws a sample of tissue with the needle.
• Biopsy at the same time as treatment — Sometimes the surgeon takes a tissue sample when the patient has surgery to remove the tumor.

Sometimes a biopsy is not possible. 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 damaging normal brain tissue. The doctor uses MRI, CT, or other imaging tests instead.

Treatment

Many people with brain tumors want to take an active part in making decisions about their medical care. They want to learn all they can about their disease and their treatment choices. However, shock and stress after a diagnosis of a brain tumor can make it hard to think of everything to ask the doctor. It often helps to make a list of questions before an appointment. To help remember what the doctor says, patients may take notes or ask whether they may use a tape recorder. Some also want to have a family member or friend with them when they talk to the doctor—to take part in the discussion, to take notes, or just to listen.

The doctor may refer the patient to a specialist, or the patient may ask for a referral. Specialists who treat brain tumors include neurosurgeons, neurooncologists, medical oncologists, and radiation oncologists. The patient may be referred to other health care professionals who work together as a team. The medical team may include a nurse, dietitian, mental health counselor, social worker, physical therapist, occupational therapist, and speech therapist. Children may need tutors to help with schoolwork.

Getting a Second Opinion

Before starting treatment, the patient might want a second opinion about the diagnosis and the treatment plan. Some insurance companies require a second opinion; others may cover a second opinion if the patient or doctor requests it.

There are a number of ways to find a doctor for a second opinion:

The patient's doctor may refer the patient to one or more specialists. At cancer centers, several specialists often work together as a team.

Preparing for Treatment

The doctor can describe treatment choices and discuss the results expected with each treatment option. The doctor and patient can work together to develop a treatment plan that fits the patient's needs.

Treatment depends on a number of factors, including the type, location, size, and grade of the tumor. For some types of brain cancer, the doctor also needs to know whether cancer cells were found in the cerebrospinal fluid.

People do not need to ask all of their questions or understand all of the answers at one time. They will have other chances to ask the doctor to explain things that are not clear and to ask for more information

Methods of Treatment

People with brain tumors have several treatment options. Depending on the tumor type and stage, patients may be treated with surgery, radiation therapy, or chemotherapy. Some patients receive a combination of treatments.

In addition, at any stage of disease, patients may have treatment to control pain and other symptoms of the cancer, to relieve the side effects of therapy, and to ease emotional problems. This kind of treatment is called symptom management, supportive care, or palliative care.

The doctor is the best person to describe the treatment choices and discuss the expected results.

A patient may want to talk to the doctor about taking part in a clinical trial, which is a research study of new treatment methods.

Surgery is the usual treatment for most brain tumors. Surgery to open the skull is called a craniotomy. It is performed under general anesthesia. Before surgery begins, the scalp is shaved. The surgeon then makes an incision in the scalp and uses a special type of saw to remove a piece of bone from the skull. After removing part or the entire tumor, the surgeon covers the opening in the skull with that piece of bone or with a piece of metal or fabric. The surgeon then closes the incision in the scalp.

Sometimes surgery is not possible. If the tumor is in the brain stem or certain other areas, the surgeon may not be able to remove the tumor without damaging normal brain tissue. Patients who cannot have surgery may receive radiation or other treatment.

Radiation therapy (also called radiotherapy) uses high-energy rays to kill tumor cells. The radiation may come from x-rays, gamma rays, or protons. A large machine aims radiation at the tumor and the tissue close to it. Sometimes the radiation may be directed to the entire brain or to the spinal cord.

Radiation therapy usually follows surgery. The radiation kills tumor cells that may remain in the area. Sometimes, patients who cannot have surgery have radiation therapy instead.

The patient goes to a hospital or clinic for radiation therapy. The treatment schedule depends on the type and size of the tumor and the age of the patient. Each treatment lasts only a few minutes.

Doctors take steps to protect the healthy tissue around the brain tumor:

• Fractionation — Radiation therapy usually is given five days a week for several weeks. Giving the total dose of radiation over an extended period helps to protect healthy tissue in the area of the tumor.
• Hyperfractionation — The patient gets smaller doses of radiation two or three times a day instead of a larger amount once a day.
• Stereotactic radiation therapy—Narrow beams of radiation are directed at the tumor from different angles. For this procedure, the patient wears a rigid head frame. An MRI or CT scan creates pictures of the tumor's exact location. The doctor uses a computer to decide on the dose of radiation needed, as well as the sizes and angles of the radiation beams. The therapy may be given during a single visit or over several visits.
• 3-dimensional conformal radiation therapy — A computer creates a 3-dimensional image of the tumor and nearby brain tissue. The doctor aims multiple radiation beams to the exact shape of the tumor. The precise focus of the radiation beams protects normal brain tissue.
• Proton beam radiation therapy — The source of radiation is protons rather than x-rays. The doctor aims the proton beams at the tumor. Protons can pass through healthy tissue without damaging it.

Chemotherapy, the use of drugs to kill cancer cells, is sometimes used to treat brain tumors. The drugs may be given by mouth or by injection. Either way, the drugs enter the bloodstream and travel throughout the body. The drugs are usually given in cycles so that a recovery period follows each treatment period.

Chemotherapy may be given in an outpatient part of the hospital, at the doctor's office, or at home. Rarely, the patient may need to stay in the hospital.

Children are more likely than adults to have chemotherapy. However, adults may have chemotherapy after surgery and radiation therapy.

For some patients with recurrent cancer of the brain, the surgeon removes the tumor and implants several wafers that contain chemotherapy. Each wafer is about the size of a dime. Over several weeks, the wafers dissolve, releasing the drug into the brain. The drug kills cancer cells.

Side Effects of Treatment

Because treatment may damage healthy cells and tissues, unwanted side effects are common. These side effects depend on many factors, including the location of the tumor and the type and extent of the treatment. Side effects may not be the same for each person, and they may even change from one treatment session to the next. Before treatment starts, the health care team will explain possible side effects and suggest ways to help the patient manage them

Surgery

Patients often have a headache or are uncomfortable for the first few days after surgery. However, medicine can usually control their pain. Patients should feel free to discuss pain relief with the doctor or nurse.

It is also common for patients to feel tired or weak. The length of time it takes to recover from an operation varies for each patient.

Other, less common, problems may occur. Cerebrospinal fluid or blood may build up in the brain. This swelling is called edema. The health care team monitors the patient for signs of these problems. The patient may receive steroids to help relieve swelling. A second surgery may be needed to drain the fluid. The surgeon may place a long, thin tube (shunt) in a ventricle of the brain. The tube is threaded under the skin to another part of the body, usually the abdomen. Excess fluid is carried from the brain and drained into the abdomen. Sometimes the fluid is drained into the heart instead.

Infection is another problem that may develop after surgery. If this happens, the health care team gives the patient an antibiotic.

Brain surgery may damage normal tissue. Brain damage can be a serious problem. The patient may have problems thinking, seeing, or speaking. The patient also may have personality changes or seizures. Most of these problems lessen or disappear with time. But sometimes damage to the brain is permanent. The patient may need physical therapy, speech therapy, or occupational therapy

Radiation Therapy

Some patients have nausea for several hours after treatment. The health care team can suggest ways to help patients cope with this problem. Radiation therapy also may cause patients to become very tired as treatment continues. Resting is important, but doctors usually advise patients to try to stay as active as they can.

In addition, radiation therapy commonly causes hair loss. Hair usually grows back within a few months. Radiation therapy also may affect the skin in the treated area. The scalp and ears may become red, dry, and tender. The health care team can suggest ways to relieve these problems.

Sometimes radiation therapy causes brain tissue to swell. Patients 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. This side effect is called radiation necrosis. Necrosis can cause headaches, seizures, or even the patient's death.

In children, radiation may damage the pituitary gland and other areas of the brain. This could cause learning problems or slow down growth and development. In addition, radiation during childhood increases the risk of secondary tumors later in life. Researchers are studying whether chemotherapy may be used instead of radiation therapy in young children with brain tumors.

Side effects may be worse if chemotherapy and radiation therapy are given at the same time. The doctor can suggest ways to ease these problems

Chemotherapy

The side effects of chemotherapy depend mainly on the drugs that are used. The most common side effects include fever and chills, nausea and vomiting, loss of appetite, and weakness. Some side effects may be relieved with medicine.

Patients who receive an implant (a wafer) that contains a drug are monitored by the health care team for signs of infection after surgery. An infection can be treated with an antibiotic.

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