Skip to main content

Rare Brain and Spine Tumors

Primary central nervous system (CNS) tumors begin in the brain or spinal cord. About 79,000 people are diagnosed a year with a primary CNS tumor and about 24,000 are malignant. View Cancer Stat Facts: Brain and Other Nervous System Cancer to see the number of new cases, lifetime risk, and people living with CNS cancers in the United States. All primary CNS cancers are rare, so often disease information, support, and expert care is hard to find.

Learn about CNS tumor anatomy, causes, symptoms, diagnosis, treatments, and who they affect to help you better understand your cancer so you can feel confident in your care. 

  • Atypical Teratoid Rhabdoid Tumor (ATRT)

    ATRTs 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • 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.

  • Medulloblastoma

    Medulloblastoma tumors are fast-growing tumors. They commonly form in the cerebellum. Learn more about medulloblastomas.

  • 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.

  • Oligodendroglioma

    Oligodendroglioma tumors can be slow- or fast-growing tumors. They occur most often in people between the ages of 35 and 44. Learn more about oligodendrogliomas and anaplastic oligodendrogliomas.

  • 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.

  • Pleomorphic Xanthoastrocytoma (PXA) and Anaplastic Pleomorphic Xanthoastrocytoma (APXA)

    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.

  • Primitive Neuro-Ectodermal Tumors (PNET)

    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, PhD; Orwa Aboud, MD; Lisa Boris, MSN; Brittany Cordeiro; Maria Fletcher Ruiz; Mark Gilbert, MD; Kristin Odom; Carlos Romo, MD; Christine Siegel, MSN; Brett Theeler, MD; Elizabeth Vera, MS; Margarita Raygada, PhD, ScM, CGC. We would also like to acknowledge Central Brain Tumor Registry of the United States (CBTRUS) for epidemiologic data on the 12 rare CNS tumor types.