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Childhood Central Nervous System Embryonal Tumors Treatment

General Information About Childhood Central Nervous System Embryonal Tumors

Central nervous system embryonal tumors begin in embryonic (fetal) cells in the brain and spinal cord.

Central nervous system (CNS) embryonal tumors form in brain cells when the fetus is beginning to develop.

The tumors may be benign (not cancer) or malignant (cancer). Most CNS embryonal tumors in children are malignant. Benign brain tumors grow and press on nearby areas of the brain. They rarely spread into other tissues. Malignant brain tumors are likely to grow quickly and spread into other brain tissue. When a tumor grows into or presses on an area of the brain, it may stop that part of the brain from working the way it should. Both benign and malignant brain tumors can cause symptoms and need treatment.

Although cancer is rare in children, brain tumors are the third most common type of childhood cancer, after leukemia and lymphoma. This summary discusses the treatment of primary brain tumors (tumors that begin in the brain). The treatment of metastatic brain tumors, which 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. See the PDQ treatment summary on Adult Brain Tumors for more information.

CNS embryonal tumors may form in different areas of the brain.

Childhood brain tumors are named based on the type of cell they formed in and where the tumor first formed in the brain.


Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.
Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic nerve, ventricles (with cerebrospinal fluid shown in blue), and other parts of the brain.

There are 6 different types of CNS embryonal tumors:

Childhood CNS atypical teratoid/rhabdoid tumor is also a type of embryonal tumor, but it is treated differently than other childhood CNS embryonal tumors. See the PDQ summary on Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment for more information.

Certain genetic conditions increase the risk of childhood CNS embryonal tumors.

Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Parents who think their child may be at risk should discuss this with the child's doctor. Risk factors for CNS embryonal tumors include having the following inherited diseases:

In most cases, the cause of CNS embryonal tumors is not known.

The symptoms of childhood CNS embryonal tumors are not the same in every child.

The following symptoms and others may be caused by a CNS embryonal tumor. Symptoms vary depending on the child's age and where the tumor is located. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

An infant or young child may be irritable or grow slowly, and may not eat well or meet developmental milestones such as sitting, walking, and talking in sentences.

Tests that examine the brain and spinal cord are used to detect (find) childhood CNS embryonal tumors.

The following tests and procedures may be used:

Childhood CNS embryonal tumors are usually diagnosed and removed in surgery.

If doctors think your child may have a CNS embryonal tumor, a biopsy may be done to remove a sample of tissue. For brain tumors, the biopsy is done by removing part of the skull and using a needle to remove a sample of tissue. Sometimes, a computer-guided needle is used to remove a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are found, the doctor may remove as much tumor as safely possible during the same surgery.

The following tests may be done on the sample of tissue that is removed:

Some pineoblastoma and pineal parenchymal tumors are diagnosed by brain imaging tests.

Sometimes, it is not possible to do a safe biopsy or completely remove the tumor in surgery, because of where it is in the brain. These tumors are diagnosed based on the results of imaging tests and other procedures.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) and treatment options depend on: