The treatment options for brain and spinal cord tumors depend on several factors, including the type of tumor and how far it has grown or spread.
Non-infiltrating astrocytomas
(Juvenile pilocytic astrocytomas, subependymal giant cell astrocytomas)
Many doctors consider these benign tumors because they tend to grow very slowly and do not grow into (infiltrate) nearby tissues. Juvenile pilocytic astrocytomas occur most often in the cerebellum in young children, while subependymal giant cell astrocytomas grow in the ventricles and are almost always seen in children with tuberous sclerosis.
In most cases, these astrocytomas can be cured by surgery alone. Radiation therapy may be given if the tumor is not completely removed, although many doctors will wait until there are signs the tumor is growing back before considering it. Even then, another operation to remove the remaining tumor may be the first option. The outlook is not as good if the astrocytoma occurs in a place that does not allow it to be removed surgically, such as the hypothalamus or brain stem.
For subependymal giant cell astrocytomas that can't be removed completely by surgery, treatment with a newer drug called everolimus (Afinitor) may shrink the tumor or slow its growth for some time. This drug is taken daily as a pill. Common side effects can include mouth sores, fever, respiratory infections, and lowered blood cell counts.
Low-grade astrocytomas
The initial treatment for these tumors is surgery when possible. Because these tumors often grow into nearby normal brain tissue, they may be hard to cure with just surgery. Usually the surgeon will try to remove as much of the tumor as safely possible. If the surgeon can remove it all this may be curative.
Radiation therapy may be given after surgery, especially if a lot of tumor remains. Otherwise, it may be postponed until the tumor starts to regrow. (Sometimes, a second surgery may be tried before giving radiation.) Radiation may also be used as the main treatment if surgery is not a good option because of where the tumor is located.
For children younger than 3, if the tumor cannot be completely removed or if it grows back, chemotherapy may be used until they are older. They may then be treated with radiation.
Intermediate- and high-grade astrocytomas
(Anaplastic astrocytomas, glioblastomas)
Although surgery is often the first treatment, these infiltrating astrocytomas are not curable by surgery. After as much of the tumor as possible is removed, radiation therapy is given, often followed by chemotherapy. For children younger than 3, radiation may be postponed until they are older. Surgery may be repeated in some cases if the tumor comes back after the initial treatment. Because these tumors are hard to cure with current treatments, clinical trials of promising new treatments may be a good option.
Oligodendrogliomas
If possible, surgery is the first option for these infiltrating tumors. Although they are usually not curable by surgery, it can relieve symptoms and prolong survival. Many of these tumors grow slowly, and surgery may be repeated if it grows back in the same spot. Chemotherapy and/or radiation therapy may be given after surgery.
If surgery is not an option, chemotherapy, with or without radiation therapy, may be helpful. These tumors may respond to chemotherapy better than other brain tumors if certain chromosome changes are in the tumor cells. You can ask your child's doctor about testing for these changes.
Ependymomas and anaplastic ependymomas
These tumors do not infiltrate normal brain tissue as extensively as astrocytomas and may sometimes be cured by surgery if the entire tumor can be removed. If some of the tumor is left behind, a second operation may be done in some cases (often after a short course of chemotherapy). Radiation therapy is recommended after surgery in most patients to try to prevent recurrence, even if it appears that all of the tumor has been removed.
The use of chemotherapy after surgery is still being tested in clinical trials. It may be recommended, but its benefit is still uncertain. It may be more helpful for anaplastic ependymomas. Very young children may be given chemotherapy to avoid or delay the use of radiation.
Sometimes the tumor cells can spread into the cerebrospinal fluid (CSF). The doctor may test the CSF for cancer cells by doing a lumbar puncture (spinal tap). Radiation may be extended to include the entire brain and spinal cord if tumor cells are found in the CSF or growing on the surface the nervous system.
Optic gliomas
These tumors invade the nerves leading to the eye. They are often hard to operate on because these nerves are very sensitive and may be harmed by surgery. Depending on where the tumor is, removing it could lead to loss of vision in one or both eyes. This is why surgery has to be considered carefully. Sometimes surgery might not be needed, because these tumors can grow very slowly.
If treatment is needed and the tumor can be completely removed without the loss of vision or other serious problems, surgery is preferred. If the patient cannot be cured with surgery, radiation therapy to this area is the preferred treatment even though it can also affect a child's vision. Often, chemotherapy may be given to younger children instead of radiation.
Brain stem gliomas
Most of these tumors are astrocytomas, although a small number are ependymomas or other tumors. These tumors usually have a characteristic appearance on MRI, so the diagnosis can often be made without surgery or a biopsy.
A small number of brain stem gliomas occur as a small tumor with very distinct edges (called a focal brain stem glioma). These tumors can often be treated successfully with surgery. In cases where surgery can't be done or doesn't completely remove the tumor, radiation therapy may be effective in slowing its growth.
Most brain stem gliomas grow throughout the brain stem, which is vital to life and can't be removed. Surgery in these cases would most likely do more harm than good, so it is usually not attempted. These brain stem gliomas are usually treated with radiation therapy. Chemotherapy is sometimes added, although it's not clear how helpful it might be. These tumors are very hard to control, and they tend to have a poor prognosis. Certain children, namely those with neurofibromatosis type I, may have a better outlook.
Primitive neuroectodermal tumors (including medulloblastoma and pineoblastoma)
Primitive neuroectodermal tumors (PNETs) are all treated in similar ways, but medulloblastomas tend to have a better outlook than other types of PNETs.
Medulloblastomas: These tumors start in the cerebellum. They tend to grow quickly and are among those most likely to spread outside of the brain (usually to the bones or the bone marrow). But they also tend to respond well to treatment. Children in the high-risk group are usually given more intensive treatment than children in the average-risk group (see "How are brain and spinal cord tumors in children staged?").
Medulloblastomas are treated with surgery followed by radiation therapy to the area where they started. High doses of radiation are aimed at the area of the tumor. Because these tumors tend to spread to the cerebrospinal fluid (CSF), lower doses of radiation may be given to the whole brain and the spinal cord (craniospinal radiation), as well. Chemotherapy is usually given after radiation therapy, and may allow doctors to use lower doses of radiation in some cases. But if the tumor has spread through the CSF, standard doses of radiation will be needed.
For children younger than 3, doctors try to use as little radiation as possible. Chemotherapy is typically the first treatment given after surgery. Depending on how the tumor responds, the chemotherapy may or may not be followed by radiation therapy.
Pineoblastomas and other PNETs: These tumors also tend to grow quickly, and they are generally harder to treat than medulloblastomas. Surgery is the main treatment for these tumors, but they are usually hard to remove completely. Still, surgery can relieve symptoms and may help other treatments to be more effective. Children 3 or older are given radiation therapy after surgery. Because these tumors tend to spread to cerebrospinal fluid (CSF), radiation therapy is often given to the whole brain and the spinal cord (craniospinal radiation).
Chemotherapy may be given with radiation therapy so that a lower dose of radiation can be used. But if the tumor has spread to the CSF, standard doses of radiation will be required. Chemotherapy is also used to treat tumor recurrence.
For children younger than 3 years, doctors try to use as little radiation as possible. Chemotherapy is typically the first treatment given after surgery. Some studies have achieved very good results using chemotherapy in young children. Depending on how the tumor responds, the chemotherapy may or may not be followed by radiation therapy.
There are some reports that giving high-dose chemotherapy followed by an autologous stem cell transplant may be an effective treatment for children with medulloblastomas or other PNETs. Several clinical trials are now studying this. For more information on stem cell transplants, see our separate document, Bone Marrow and Peripheral Blood Stem Cell Transplant.
Meningiomas
Surgery is the main treatment for these tumors. Children are usually cured if the surgery completely removes the tumor. Some tumors, particularly those at the base of the brain, cannot be removed completely, and some are malignant and come back in spite of apparent complete removal. Radiation therapy may control the growth of meningiomas that cannot be removed completely or those that come back after surgery. Chemotherapy may be tried if surgery and radiation aren't effective, but it is not helpful in many cases.
Schwannomas (including acoustic neuromas)
These slow-growing tumors are usually benign and are effectively cured by surgical removal. In some centers, small vestibular schwannomas (also known as acoustic neuromas) are treated by stereotactic radiosurgery (see the section, "Radiation therapy"). For the rare malignant schwannoma, radiation therapy is often given after surgery.
Spinal cord tumors
These tumors are usually treated similarly to those of the same type in the brain. Astrocytomas of the spinal cord usually cannot be completely removed. They may be treated with surgery to remove as much tumor as possible, followed by radiation therapy, or with radiation therapy alone. Chemotherapy may be used in younger children or if the tumor appears to be aggressive.
Meningiomas near the spinal cord are often cured by surgical removal. Some ependymomas can be cured by surgery as well. If an ependymoma cannot be completely removed, radiation therapy will follow surgery.
Choroid plexus tumors
Benign choroid plexus papillomas are usually cured just with surgery. Choroid plexus carcinomas are malignant tumors that are only sometimes cured by surgery. After surgery, these carcinomas are usually treated with radiation and/or chemotherapy.
Craniopharyngiomas
Craniopharyngiomas can be treated by surgically removing most of the tumor (debulking) followed by radiation in most cases. Partial surgical removal followed by very focused radiation therapy may cause fewer severe side effects than complete removal.
Germ cell tumors
The most common germ cell tumor, germinoma, is usually cured by radiation therapy alone. Chemotherapy may be added if the tumor is very large or if radiation doesn't destroy it completely. In very young children, chemotherapy may be used instead of radiation therapy. If other types of germ cell tumors are present, either mixed or not mixed with germinoma, the outlook is usually not as good.
Other types of germ cell tumors (such as teratomas and yolk sac tumors) are usually treated with both radiation therapy and chemotherapy. Sometimes these tumors spread to the cerebrospinal fluid (CSF), and radiation therapy to the spinal cord and brain is needed.
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