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Brain Stem Glioma (Childhood) General Information What is childhood brain stem glioma? Childhood brain stem glioma is a disease in which benign (noncancer) or malignant (cancer) cells form in the tissues of the brain stem. The brain stem is the part of the brain connected to the spinal cord. It is located in the lowest part of the brain, just above the back of the neck. The brain stem is the part of the brain that controls breathing, heart rate, and nerves and muscles used in seeing, hearing, walking, talking, and eating. Although cancer is rare in children, brain tumors are the most common type of childhood cancer other than leukemia or lymphoma. This summary refers to the treatment of primary brain tumors (tumors that begin in the brain). Treatment for metastatic brain tumors, which are secondary tumors formed by cancer cells that begin in other parts of the body and spread to the brain, is not discussed in this summary. Brain tumors can occur in both children and adults; however, treatment for children may be different than treatment for adults. (Refer to Adult Brain Tumor Treatment for more information.) The cause of most childhood brain tumors is unknown. Tests that examine the brain are used to detect (find) and diagnose childhood brain stem glioma. The following tests and procedures may be used: CT scan (CAT scan): A CT scan creates a series of detailed pictures of areas inside the body, taken from different angles. The pictures are created by a computer linked to an x-ray machine. This test is also called computed tomography, computerized tomography, or computerized axial tomography. MRI (magnetic resonance imaging): A procedure in which a magnet linked to a computer is used to create detailed pictures of areas inside the body. This test is also called nuclear magnetic resonance imaging (NMRI). Biopsy: Cells, tissues, or fluid are removed and viewed under a microscope to see if cancer cells are present. Sometimes a brain tumor biopsy is done by making a small hole in the skull and using a needle to remove a sample of tumor tissue. A biopsy will not be done if the tumor cannot be safely reached or if it has spread widely throughout the brain stem. Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery) depends on the type of brain stem glioma, where it is located within the brain stem, whether it has spread within the brain stem, and whether or not the child has an underlying condition called neurofibromatosis type 1. Treatment options depend on the type and location of the tumor. Stages Staging is the process used to find out how far the cancer has spread. There is no standard staging system for childhood brain stem glioma. The tumors are described by type: -Diffuse intrinsic glioma is a tumor that has spread widely throughout the brain stem. -Focal or low-grade glioma is a tumor that is localized to one area of the brain stem. Recurrent Childhood Brain Stem Glioma Recurrent childhood brain stem glioma is a tumor that has recurred (come back) after it has been treated. If childhood brain stem glioma recurs, it may do so many years after initial treatment. The tumor may come back in the brain or in other areas of the central nervous system. Treatment Option Overview Four types of standard treatment are used: -Surgery -Radiation therapy -Chemotherapy -Cerebrospinal fluid diversion -Other types of treatment are being tested in clinical trials. -Radiation therapy with radiosensitizers There are different types of treatment for children with brain stem glioma. Different types of treatment are available for children with brain stem glioma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the "standard" treatment, the new treatment may become the standard treatment. Because cancer in children is rare, taking part in a clinical trial should be considered. Clinical trials are taking place in many parts of the country. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team. Children with brain stem glioma should have their treatment planned by a team of doctors with expertise in treating childhood brain tumors. Your child's treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist may refer you to other pediatric doctors who have experience and expertise in treating children with brain tumors and who specialize in certain areas of medicine. These may include the following specialists: Neurosurgeon. Neurologist. Neuropathologist. Neuroradiologist. Rehabilitation specialist. Radiation oncologist. Medical oncologist. Endocrinologist. Psychologist. Four types of standard treatment are used: Surgery Surgery is used when possible to treat childhood brain stem gliomas. Depending on the location of the tumor, and whether it has spread widely within the brain stem, your child's doctor may perform a craniotomy (an operation in which an opening is made in the skull) to remove as much of the tumor as possible. Radiation therapy Radiation therapy is the use of x-rays or other types of radiation to kill cancer cells and shrink tumors. Radiation therapy may use external radiation (using a machine outside the body) or internal radiation. Internal radiation involves putting radioisotopes (materials that produce radiation) through thin plastic tubes into the area where cancer cells are found. Hyperfractionated radiation therapy is a way of giving radiation therapy in smaller-than-usual doses two or three times a day instead of once a day. External radiation is the type usually used to treat childhood brain tumors. Radiation therapy may be used alone or in addition to chemotherapy. Chemotherapy Chemotherapy is the use of drugs to kill cancer cells. Chemotherapy may be taken by mouth, or it may be put into the body by inserting a needle into a vein or muscle. Either type of chemotherapy is called systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body. Because radiation therapy can affect growth and brain development in young children, clinical trials are studying ways of using chemotherapy to delay or reduce the need for radiation therapy. Cerebrospinal fluid diversion Cerebrospinal fluid diversion is a process used to drain fluid that has built up around the brain and spinal cord. A shunt (a long, thin tube) is placed in a ventricle (fluid-filled cavity) of the brain and threaded under the skin to another part of the body, usually the abdomen. The shunt acts as a drainpipe to carry excess fluid away from the brain so it may be absorbed elsewhere in the body. Other types of treatment are being tested in clinical trials. Radiation therapy with radiosensitizers Radiosensitizers are drugs that make tumor cells more sensitive to radiation. Combining radiation with radiosensitizers may kill more tumor cells. This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Treatment Options For Untreated Childhood Brain Stem Glioma Untreated childhood brain stem glioma is a tumor that has not been treated except to relieve symptoms caused by the tumor. Treatment of diffuse intrinsic brain stem glioma may include the following: -Radiation therapy. -A clinical trial of chemotherapy combined with radiation therapy. -A clinical trial of chemotherapy to delay the use of radiation therapy. Treatment of focal or low-grade glioma may include the following: -Surgery. -Cerebrospinal fluid diversion followed by careful observation (watchful waiting without immediate treatment). Watchful waiting is closely monitoring a patient's condition but withholding treatment until symptoms appear or change. -A clinical trial of surgery followed by watchful waiting. Treatment of brain stem glioma in children with neurofibromatosis type 1 may be watchful waiting. The tumors are slow-growing in these children and may not need specific treatment for years. This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Treatment Options for Recurrent Childhood Brain Stem Glioma Treatment of recurrent childhood brain stem glioma depends on the type of tumor, whether it comes back in the place in which it originated or in another part of the brain, and the type of treatment previously given. Treatment of recurrent diffuse intrinsic brain stem glioma may include the following: -Surgery. -Palliative therapy to relieve symptoms and improve quality of life. -A clinical trial of a new therapy. Treatment of recurrent focal or low-grade childhood brain stem glioma may include the following: -Surgery. -Chemotherapy. The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals. The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation. The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. 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