Ependymoma Overview

Ependymoma Overview

What is ependymoma?

Ependymoma is a rare tumor of the brain or spinal cord. It occurs in both adults and children.

Ependymoma is a primary tumor, which means that it starts in either the brain or spine. The brain and spine are part of the central nervous system (CNS). Primary brain and spinal cord tumors are typically grouped by where the cells start.

The most common types of cells in the central nervous system are neurons and glial cells. Tumors from neurons are rare. Glial cells are the cells that support the brain. Tumors  that occur from these cells are called gliomas.

Glial cell subtypes of the CNS include:

  • Astrocytes
  • Oligodendrocytes
  • Ependymal cells

CNS brain tumors associated with all three types of glial cells are recognized by the World Health Organization as astrocytomas, oligodendrogliomas and ependymomas.

How do ependymomas form?

Scientists believe they develop from precursor cells to the ependymal cells. These cells line the ventricles (fluid-filled spaces in the brain) and the central canal of the spinal cord.

The tumor can occur anywhere in the CNS, including the brain and the spinal cord. The cause of ependymoma is not known.

VentriclesDo they spread?

Sometimes, tumor cells can spread in the cerebrospinal fluid (CSF). They may spread to one or multiple areas in the brain, spine, or both.

Although it is rare, the tumor can spread to other parts of the body.

 

Who does ependymoma effect?

Ependymomas can occur in both children and adults. It can occur in males slightly more often than females. It also occurs in white people more often than those of other ethnicities.

Approximately 2,100 people per year are diagnosed with ependymoma in the United States (according to a CBTRUS report).

Pediatric ependymoma  How often do ependymomas form in children?

While there are a fewer number of children diagnosed than adults each year, among the children diagnosed each year, it is more common among the pediatric community than the adult community.

Ependymomas are the third most common form of childhood brain and spine tumors. Most occur in young infants and children. The majority occur in the brain. Often they are seen in the back part of the brain (the posterior fossa).

  • In children aged 0-14 years, ependymomas accounted for 5.6% of all tumors diagnosed. Approximately 280 children are diagnosed per year.
  • In children aged 15-19 years, ependymoma accounted for 4.5% of all tumors diagnosed. Approximately 50 teenagers are diagnosed per year.
  • The incidence in children (aged 0-19 years) was similar in males and females. The incidence of variants is higher in males than females.

Adult ependymoma  How often do ependymomas form in adults?

Ependymomas account for 5 percent of adult gliomas. The majority occur in the spine.

  • In adults 20+ years, ependymomas accounted for 1.9% of all tumors diagnosed. Approximately 1,800 adults are diagnosed per year.
  • Ependymomas/ anaplastic ependymomas occur with similar incidence in males and females. The incidence of variants is higher in males than females. The incidence of both ependymomas/anaplastic ependymomas and variants was higher in whites than blacks.

CERN has the largest database of adult and pediatric ependymoma tissue. We are evaluating differences based on grade, location and symptom presentation, to improve our understanding and to improve diagnosis and our understanding of the prognosis.

Currently, the Surveillance Epidemiology and the End Results (SEER) Program and the Central Brain Tumor Registry (CBTRUS) group all grades of ependymomas together for reporting purposes.