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.

Atypical Teratoid/Rhabdoid Tumor (AT/RT): Diagnosis and Treatment

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.
Choroid Plexus Tumor: Diagnosis and Treatment

Choroid plexus tumors can be slow- or fast-growing. They arise from a structure in the brain called the choroid plexus.
Diffuse Midline Glioma: Diagnosis and Treatment

Diffuse midline gliomas are fast-growing tumors. They form in the brainstem, thalamus, spinal cord, or cerebellum.
Ependymoma: Diagnosis and Treatment

Ependymomas can form anywhere in the CNS. They often occur near the ventricles in the brain and the central canal of the spinal cord.
Gliomatosis Cerebri: Diagnosis and Treatment

Gliomatosis cerebri refers to a diffuse pattern of glioma cells with extensive growth that invade multiple lobes of the brain. They are most common in older adults.
Gliosarcoma: Diagnosis and Treatment

Gliosarcomas are fast-growing tumors. They are commonly found along the surface of the brain but can also form in the spinal cord.
Medulloblastoma: Diagnosis and Treatment

Medulloblastomas are fast-growing tumors commonly found in the cerebellum. In adults, this tumor typically affects those between 20 and 40 years of age.
Meningioma: Diagnosis and Treatment

Meningiomas are the most common type of primary brain tumor. Higher grade meningiomas are very rare, but they are fast-growing tumors.
Oligodendroglioma and Other IDH-Mutated Tumors: Diagnosis and Treatment

Oligodendrogliomas can be slow- or fast-growing tumors. Diagnosing an oligodendroglioma requires finding two genetic alterations: an IDH mutation and a 1p19q codeletion.
Pineal Region Tumors: Diagnosis and Treatment

Pineal region tumors occur in the middle of the brain in the pineal region. They can also spread to other areas in the CNS.
Pleomorphic Xanthoastrocytoma (PXA) and Other BRAF-Altered Tumors: Diagnosis and Treatment

Pleomorphic xanthoastrocytoma (PXA) tumors can be slow- or fast-growing tumors. They occur most often between the ages of 15 and 39.
Primitive Neuro-Ectodermal Tumors (PNETs): Diagnosis and Treatment

Primitive neuro-ectodermal tumors (PNETs) are very rare, fast-growing tumors. The tumors in this group are currently being reclassified and given other specific names.

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.