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People with this condition tend to develop benign or cancerous tumors in different parts of the body, including hemangioblastomas (benign blood vessel tumors) in the brain, spinal cord, or retina, as well as tumors of the inner ear, kidney, adrenal gland, and pancreas. Most often the gene changes are inherited, but in some cases the changes happen before birth in people whose parents don’t have them.
People with this condition are at higher risk for developing gliomas, along with breast cancer, soft tissue sarcomas, leukemia, adrenal gland cancer, and certain other types of cancer.
People with this condition may have subependymal giant cell astrocytomas (SEGAs), which are low-grade astrocytomas that develop beneath the ependymal cells of the ventricles.
They may also have other benign tumors of the brain, skin, heart, kidneys, and other organs.
Some of these families have well-defined disorders, such as: This genetic disorder, also known as von Recklinghausen disease, is the most common syndrome linked to brain or spinal cord tumors.This condition, which is much less common than NF1, is associated with vestibular schwannomas (acoustic neuromas), which almost always occur on both sides of the head.It is also linked with an increased risk of meningiomas or spinal cord ependymomas.Radiation-induced tumors are still fairly rare, but because of the increased risk (as well as the other side effects), radiation therapy is only given to the head after carefully weighing the possible benefits and risks.For most patients with other cancers involving the brain or head, the benefits of radiation therapy far outweigh the risk of developing a brain tumor years later.
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The possible risk from exposure to imaging tests that use radiation, such as x-rays or CT scans, is not known for sure.