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Detailed Guide: Brain / CNS Tumors in Children
What Are the Risk Factors for Brain and Spinal Cord Tumors in Children?

A risk factor is anything that affects a person's chance of getting a disease such as cancer. Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. But risk factors don't tell us everything. Having a risk factor, or even several, does not mean that a person will get the disease, and many people who have no known risk factors get cancer.

Unlike many adult cancers, lifestyle-related risk factors do not seem to play a large role in childhood cancers. On top of this, very few risk factors for brain tumors have been found. There is no clear cause for most brain tumors.

Environmental risk factors

The only well-established environmental risk factor for brain tumors is radiation exposure to the head, which most often comes from the treatment of other conditions. For example, before the risks of radiation were well known (more than 50 years ago), children with ringworm of the scalp (a fungal infection) often received low-dose radiation therapy. This was found to increase their risk of brain tumors later in life. Today, most radiation-caused brain tumors are the result of radiation to the head given to treat other cancers such as certain leukemias.

Inherited and genetic conditions

In rare cases (less than 1 in 10 brain tumors), children may have inherited abnormal copies of genes from a parent that put them at increased risk for certain types of brain tumors. In some cases, the abnormal genes are not inherited but occur as a result of changes (mutations) in the gene before birth.

People with inherited tumor syndromes often have many tumors that start when they are young. Some of the more well-known disorders are listed below.

Neurofibromatosis type 1 (von Recklinghausen disease): This is the most common inherited cause of brain or spinal cord tumors. Children with this syndrome may have optic gliomas or other gliomas of the brain or spinal cord, or neurofibromas (benign tumors of peripheral nerves). Changes in the NF1 gene cause this disorder.

Neurofibromatosis type 2: Less common than von Recklinghausen disease, this condition is associated with cranial or spinal nerve schwannomas, especially acoustic neuromas. It is also linked to an increased risk of meningiomas, as well as spinal cord gliomas or ependymomas. Changes in the NF2 gene are responsible for neurofibromatosis type 2.

Tuberous sclerosis: This condition may be associated with subependymal giant cell astrocytomas, as well as with other benign tumors of the brain, skin, heart, or kidneys. It is caused by changes in either the TSC1 or the TSC2 gene.

Von Hippel-Lindau disease: Children with this disease have an inherited tendency to develop hemangioblastomas (blood vessel tumors) of the cerebellum, spinal cord, or retina, as well as kidney cancer. It is caused by changes in the VHL gene.

Li-Fraumeni syndrome: People with this syndrome have an increased risk of gliomas, as well as breast cancer, soft tissue sarcomas, leukemia, and adrenal gland cancers. It is caused by changes in the p53 gene.

Other inherited conditions, including Gorlin syndrome, Turcot syndrome, Cowden syndrome, and hereditary retinoblastoma are also linked with increased risks of certain types of brain and spinal cord tumors. Some families may have genetic disorders that are not well recognized or that may even be unique to a particular family.

Last Medical Review: 04/07/2008
Last Revised: 05/13/2009

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