What is ependymoma?
Ependymoma is a rare type of primary brain or spinal cord tumor. Primary brain and spinal cord tumors are a type of tumor that starts in the central nervous system (CNS).
These tumors are classified according to the presumed cell of origin. The most common types of cells in the nervous system are neurons and glial cells, although tumors from neurons are rare. Glial cells are the supportive cells of the brain and tumors arising from these cells are called gliomas. Subtypes of glial cells of the CNS include:
Astrocytes cells
- Oligodendrocytes cells
- Ependymal cells
The World Health Organization (WHO) Classification of Tumors of the CNS recognizes brain tumors that arise from all three types of glial cells, such as astrocytomas, oligodendrogliomas and ependymomas.
How do ependymomas form? Do they spread?
Ependymomas are a rare type of glioma that are thought to develop from the ependymal cells that line the ventricles (fluid-filled spaces in the brain) and the central canal of the spinal cord. Ependymomas can occur anywhere in the CNS, including the brain or the spinal cord. Occasionally, ependymoma tumor cells can spread in the cerebrospinal fluid (CSF) and involve multiple areas. Although it is very rare for ependymoma to spread to other parts of the body, the development of tumor cells into the CSF often results in tumors developing in other areas of the CNS. Typically, tumor nodules form where the tumor cells settle such as the base of the brain and the bottom of the spinal cord.
Who does ependymoma effect?
Ependymomas represent three to six percent of all CNS tumors. They can occur in both children and adults.
How often do ependymomas form in children?
The incidence of ependymomas is higher in children than in adults. Ependymomas are the third most common form of pediatric brain tumors of the CNS, and the majority of these tumors occur in young patients’ brains. Specifically, these tumors are most commonly found in the posterior fossa.
How often do ependymomas form in adults?
For adults, ependymomas account for five percent of adult gliomas with the majority occurring in the spine.
Currently, the Surveillance Epidemiology and the End Results (SEER) Programe and the Central Brain Tumor Registry (CBTRUS) group all grades of ependymomas together for reporting purposes.
According to a CBTRUS report on incidence rates between 1998 and 2002, 1,126 ependymomas and anaplastic ependymomas were diagnosed for an adjusted rate per 100,000 person-years of 0.26. This rate is slightly higher in males (0.29) than females (0.22), and in whites (0.27) versus blacks (0.12).
The CERN Foundation is currently evaluating the data from SEER and CBTRUS to look for incidence on survival and incidence based on tumor grade. In addition, we are compiling one of the largest databases of adult ependymoma tissue to evaluate differences based on grade, location and symptom presentation.