Brain and Spinal Cord Tumors in Children

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Treating Brain/CNS Tumors In Children TOPICS

Treatment of specific types of childhood brain and spinal cord tumors

The treatment options for brain and spinal cord tumors depend on many factors, including the type of tumor and how far it has grown or spread.

Non-infiltrating astrocytomas
(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. Pilocytic astrocytomas occur most often in the cerebellum in young children, while subependymal giant cell astrocytomas (SEGAs) grow in the ventricles and are almost always seen in children with tuberous sclerosis.

Most children with these astrocytomas can be cured by surgery alone. They may be given radiation therapy 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 SEGAs that can’t be removed completely by surgery, treatment with the drug everolimus (Afinitor) may shrink the tumor or slow its growth for some time. This drug is taken daily as a pill.

Low-grade astrocytomas
(Fibrillary or diffuse astrocytomas)

The initial treatment for these tumors is surgery if it can be done, or biopsy to confirm the diagnosis if surgery is not feasible. 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, the child may be cured.

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 to try to slow the tumor growth until they are older. They may then be treated with radiation.

Intermediate- and high-grade astrocytomas
(Anaplastic astrocytomas, glioblastomas)

Surgery is often the first treatment for these infiltrating astrocytomas if it can be done, but these tumors are almost never cured by surgery. In some cases only a needle biopsy is safe. When surgery is used, as much of the tumor as possible is removed, and then radiation therapy is given, often followed by chemotherapy. If surgery can’t be done, radiation is the main treatment, again often followed by chemotherapy.

If the child is 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 the tumor cells have certain chromosome changes. 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 might 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 tumor 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 start in the optic nerves (the nerves leading from the eyes to the brain). They are often hard to operate on because these nerves are very sensitive and might be harmed by surgery. Depending on where the tumor is, removing it could lead to loss of vision in one or both eyes, so the benefits and risks of surgery have to be considered carefully. In some cases, a child might have already lost some vision because of the tumor itself. Sometimes surgery might not be needed, because these tumors can grow very slowly.

If treatment is needed and the tumor can be completely removed, surgery is often preferred. But in many cases (especially if the child has neurofibromatosis type 1) the tumor is likely to have spread too far along the optic nerves to be removed completely with surgery. Radiation therapy is preferred for these tumors if treatment is needed, although it can also affect a child’s vision.

Younger children may be given chemotherapy instead of radiation. Radiation may then be used if needed as the child gets older.

Brain stem gliomas

Most of these tumors are astrocytomas, although a small number are ependymomas or other tumors. These tumors usually look a certain way on MRI, so the diagnosis can often be made without surgery or a biopsy.

Focal brain stem gliomas: A small number of brain stem gliomas occur as a small tumor with very distinct edges (called a focal brain stem glioma). Some of these tumors grow so slowly that treatment might not be needed unless the tumor is causing problems. If treatment is needed, these tumors can often be treated successfully with surgery. If surgery can’t be done, radiation therapy may be used to slow its growth. Radiation may also be used if surgery doesn’t completely remove the tumor.

Diffuse brain stem gliomas: Most brain stem gliomas grow diffusely throughout the brain stem, rather than growing as a distinct (focal) tumor. These tumors often start in the pons, where they are called diffuse intrinsic pontine gliomas. The brain stem is vital to life and can’t be removed, so surgery in these cases would most likely do more harm than good, and it is usually not attempted. Diffuse brain stem gliomas typically are treated with radiation therapy. Chemotherapy is sometimes added, although it’s not clear how helpful it might be.

Diffuse tumors are very hard to control, and they tend to have a poor prognosis (outlook). But in children with neurofibromatosis type 1, these tumors often grow slowly (or even stop growing), so these children tend to have a better outlook. Because these tumors are hard to treat, clinical trials of promising new treatments may be a good option.

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 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 standard-risk group (see “How are brain and spinal cord tumors in children staged?”).

Medulloblastomas are treated with surgery when possible, 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), children 3 or older also may be given lower doses of radiation to the whole brain and the spinal cord (craniospinal radiation). 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 effective 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, Stem Cell Transplant (Peripheral Blood, Bone Marrow, and Cord Blood Transplants).

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 these tumors. 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 cured by surgery. 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 removed completely. 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 after surgery instead of radiation in younger children. It may also be used after radiation therapy in older children if the tumor appears to be growing quickly.

Meningiomas near the spinal cord are often cured by surgery. Some ependymomas can be cured by surgery as well. If an ependymoma cannot be completely removed, radiation therapy will be given after 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 grow very close to the pituitary gland, the optic nerves, and blood vessels that supply the brain, so they can be hard to remove completely without causing side effects. Some neurosurgeons prefer to remove as much of the tumor as possible, while others prefer to remove most of the tumor (debulking) and then give radiation therapy.

Partial surgical removal followed by very focused radiation therapy may cause fewer severe side effects than complete removal, but it is not yet clear if this approach is as good at preventing the tumor from growing back.

Germ cell tumors

The most common germ cell tumor, germinoma, can usually be cured by radiation therapy alone (after the diagnosis is made by surgery or study of a cerebrospinal fluid sample). Chemotherapy may be added if the tumor is very large or if radiation doesn’t destroy it completely. To try to reduce side effects in children who have not yet reached puberty, some doctors are now using chemotherapy followed by a reduced dose of radiation as the main treatment. 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 rarely cured by surgery. Both radiation therapy and chemotherapy are used in their treatment, but in some cases this might not control the tumor completely. Sometimes these tumors spread to the cerebrospinal fluid (CSF), and radiation therapy to the spinal cord and brain is needed as well.


Last Medical Review: 03/22/2013
Last Revised: 01/31/2014