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About Ependymomas

Ependymomas are brain tumors that arise from ependymal cells. The most common surgical treatment for ependymoma is craniotomy and surgical removal, and the most common nonsurgical treatment is stereotactic radiosurgery.

Ependymomas, a type of glioma, are uncommon, accounting for 1.9 percent of primary brain tumors among adults and 9 percent among children. Ependymomas can arise in the brain or spine. For information about spinal ependymomas, please click here.

When these tumors occur in the brain, they are typically described as arising in either the supratentorial or infratentorial region of the brain. The terms supratentorial and infratentorial describe each region in relation to the tentorium.

  • The supratentorial region makes up the upper two-thirds of the brain. It includes the cerebrum and the lateral and third ventricles.
  • The infratentorial region makes up the lower third of the brain. It includes the brainstem, cerebellum and fourth ventricle.

Ependymomas are assigned a grade that reflects the appearance of the tumor cells under a microscope. Grade I designates a typical tumor cell appearance, Grade II a slightly atypical tumor cell appearance and Grade III a significantly atypical tumor cell appearance.

Ependymomas are broken down into the following major types:

  • Subependymoma (Grade I): This type is slow-growing and benign; it arises near a ventricle.
  • Myxopapillary ependymoma (Grade I): This type is usually slow-growing and benign; it is found in the spine.
  • Ependymoma (Grade II): The most common, this type is typically benign and found along the ventricles in either the infratentorial region or the spine. This type is further categorized—on the basis of how the cells look under a microscope—as papillary ependymoma, clear cell ependymoma or tanycytic ependymoma.
  • RELA fusion–positive (Grade II or III): This is a new designation, added in 2016. This type is characterized by a molecular abnormality, RELA, that promotes tumor formation and growth. This tumor type is found in the supratentorial region.
  • Anaplastic ependymoma (Grade III): These are the fastest growing ependymomas and are malignant. They tend to occur in the infratentorial region.

Subependymomas and myxopapillary ependymomas, the Grade I tumors, are easier to treat than the Grade II and Grade III tumors, which tend to recur after initial treatment. However, in general, ependymal tumors tend to not invade nearby tissue but instead displace it as they grow, making surgical removal of many of these tumors possible.

About 10 to 15 percent of ependymomas metastasize, via the cerebrospinal fluid, to other areas in the central nervous system. It is rare for an ependymoma to metastasize outside the central nervous system.

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