Brain Tumors and Central Nervous System Tumors in Children


Brain tumors in children occur in the central nervous system (CNS) and spinal cord. Together, these important organs control functions necessary to sustain life, such as breathing, heart rate, movement, thinking and learning. Important substances produced in the brain stimulate and control many other organs in the body. When pediatric brain tumors develop, they have many unique properties and symptoms.

There are several different types of brain tumors. Collectively, they comprise nearly 20% of childhood cancers diagnoses. In general, brain tumors tend NOT to "metastasize" or spread to distant areas of the body outside the brain or spinal cord. They can recur locally, however, a condition where they spread to other areas of the central nervous system.

Common types of brain tumors include:

Nick Wolf
Nick Wolf
Primitive Neuroectodermal Tumor (PNET)
6 Years Old at Diagnosis
Read Nick's Story 

Medulloblastoma (also called PNET or Primitive Neuroectodermal Tumor)

Medulloblastoma is the most common malignant brain tumor in children. It is a fast growing tumor that begins in the lower back part of the brain (the posterior fossa) and can spread to other parts of the body. In up to one-third of patients, the tumor will have spread to the brain lining (meninges) and spinal cord at the time of diagnosis. These children often have vomiting, headache and balance problems at diagnosis and may need shunts placed to relieve obstruction of spinal fluid.

Supratentorial PNET and Pineoblastomas

These tumors typically are located in the upper part of the brain. Due to the location of the tumor, the patient’s initial problems may include the onset of seizures or eye abnormalities, as well as problems with headache, nausea and vomiting. These PNETs have a similar pathology to medulloblastomas and are treated similarly.

Gliomas (sometimes referred to as Astrocytoma)

Gliomas are brain tumors that begin in the glial cells, which are cells that surround and support the proper functioning of nerve cells. Astrocytomas are brain tumors that form in astrocyte cells, which are a specific type of glial cell. About half of brain tumors in children are astrocytomas. As most gliomas originate from astrocytes, the terms are often used interchangeably.

Gliomas are classified as either low-grade or high-grade based on how likely they are to grow and spread. Low-grade gliomas usually are slow growing and stay in a local area of the brain. High-grade gliomas grow quickly and spread easily throughout the brain.

High-grade gliomas are the most common lethal tumors in adults, but also occur in children. Due to the location of the tumor, these patients may have seizures and significant paralysis at the time of diagnosis. Because high-grade gliomas are much more aggressive, they require more intensive treatments.

Brain Stem Glioma

These high-grade gliomas grow amidst the nerves coming down the upper spine. Children may bediagnosed with eye movement problems, imbalance, paralysis and swallowing difficulties. There are some children who survive, but this is an extremely difficult tumor to treat.

Ependymoma

Ependymoma (grade II), and the more malignant ependymoblastoma (grade III), tend to occur in children under 6 years old. These tumors usually are located in the lower back part of the brain (posterior fossa), but may also arise higher in the brain or in the spinal cord. As with medulloblastoma, children with posterior fossa tumors tend to be diagnosed because of vomiting, headache and/or balance problems.

Germ Cell Tumors

Germ cell tumors are rare and occur most commonly in the pituitary or pineal glands just behind the eyes. In this location, it is usually possible to biopsy the tumor but not remove it. Germ cell tumors arise from the same kind of cells that exist in the ovaries and testes. There are several types of germ cell tumors, including pure germinomas and non-germinomatous germ cell tumors like yolk sac tumors, embyonal cell carcinomas, choriocarcinoma, mixed tumors, and malignant teratomas.

Spinal Cord Tumors

There are several types of spinal cord tumors including ependymomas, and low- and high-grade astrocytomas. Treatment often includes removing as much of the tumor as possible without further damaging the spinal nerves. For low-grade tumors, some children also receive outpatient chemotherapy similar to that used for low-grade astrocytomas. For high-grade spinal cord tumors, children are generally also treated with chemotherapy and radiation to the tumor.

Other Rare Brain Tumors

Choroid Plexus Tumors, including papillomas and carcinomas, usually occur in infants and are treated primarily with surgical resection and occasionally chemotherapy. Craniopharyngiomas are treated by surgical resection and radiation therapy based on the location of the tumor and involvement of the surrounding tissues. Sarcomas of various types that usually arise from soft tissues in the body, including muscle, sometimes occur in the brain. These sarcomas are treated with maximal surgery, local radiation and chemotherapy appropriate for body sarcomas.

Last updated June, 2014

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