Rare Brain and Spine Tumors
Primary central nervous system (CNS) tumors begin in the brain or spinal cord. There are over 130 different CNS tumor types. However, they account for less than 2 percent of all cancers diagnosed each year in the United States. For more statistics, visit the NCI Surveillance, Epidemiology, and End Results Program website.
Because primary CNS cancers are so rare, disease information, support, and expert care can be hard to find. NCI-CONNECT started with 12 select tumor types, each with fewer than 2,000 people diagnosed per year in the United States. It has since expanded its selection to include tumor subtypes based on molecular findings—and will continue to do so as new discoveries are made. Explore these NCI-CONNECT resources to learn about CNS anatomy, as well as CNS tumor causes, symptoms, diagnosis, and treatment.
Rare Brain and Spine Tumors
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Atypical Teratoid/Rhabdoid Tumor (AT/RT)
AT/RTs are very rare, fast-growing tumors that often occur in the brain and spread to the spinal cord. They are caused by changes in a gene known as SMRCB1. Learn more about ATRTs.
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Choroid Plexus Tumors
Choroid plexus tumors can be slow- or fast-growing tumors. They arise from a structure in the brain called the choroid plexus. Learn more about choroid plexus tumors.
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Diffuse Midline Gliomas
Diffuse midline gliomas are fast-growing tumors. They form in the brainstem, thalamus, spinal cord, and cerebellum. Learn more about brainstem and diffuse midline gliomas.
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Ependymoma
Ependymoma tumors are more common in the spine than the brain in adults. They often occur near the central canal of the spinal cord and ventricles of the brain. Learn more about ependymomas.
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Gliomatosis Cerebri
Gliomatosis cerebri tumors arise from glial cells in the brain. They often come back after treatment and are fast-growing. Learn more about gliomatis cerebri tumors.
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Gliosarcoma
Gliosarcoma tumors are fast-growing tumors. They are commonly found along the surface of the brain from glial cells. Learn more about gliosarcoma tumors.
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Medulloblastoma
Medulloblastoma tumors are fast-growing tumors. They commonly form in the cerebellum. Learn more about medulloblastomas.
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Meningioma
Meningioma tumors are the most common type of primary brain tumor, but higher grade meningiomas are very rare, fast-growing tumors. Learn more about atypical and anaplastic meningiomas.
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Oligodendroglioma and Other IDH-Mutated Tumor
Oligodendroglioma tumors can be slow- or fast-growing tumors. They occur most often in people between the ages of 35 and 44.
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Pineal Region Tumors
Pineal region tumors occur in the middle of the brain in the pineal region. They can spread to other areas in the central nervous system. Learn more about pineal region tumors.
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Pleomorphic Xanthoastrocytoma (PXA) and Other BRAF-Altered Tumors: Diagnosis and Treatment
Pleomorphic xanthoastrocytoma (PXA) and anaplastic pleomorphic xanthoastrocytoma (APXA) tumors can be slow- or fast-growing tumors. They occur most often in young adulthood. Learn more about PXAs and APXAs.
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Primitive Neuro-Ectodermal Tumors (PNETs)
Primitive Neuro-Ectodermal Tumors (PNET) are very rare, fast-growing tumors. PNETs is a group of tumors that form in the ectoderm, the outermost layer of cells of an embryo in early development. Learn more about PNETs.
We would like to acknowledge the following authors who have contributed to writing and reviewing content included on the 12 rare CNS tumor types highlighted on the NCI-CONNECT website: Terri Armstrong, Ph.D.; Orwa Aboud, M.D.; Lisa Boris; Brittany Cordeiro; Maria Fletcher Ruiz; Mark Gilbert, M.D.; Kristin Odom; Carlos Romo, M.D.; Christine Siegel; Brett Theeler, M.D.; Elizabeth Vera; Margarita Raygada, Ph.D; Marta Penas-Prado, M.D.; Byram Ozer, M.D., Ph.D.; and Raleigh McElvery. We would also like to acknowledge the Central Brain Tumor Registry of the United States (CBTRUS) for epidemiological data on the 12 rare CNS tumor types.