FAQs About Ependymoma

What is ependymoma?

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. CNS tumors associated with all three types of glial cells are recognized by the World Health Organization as astrocytomas, oligodendrogliomas and ependymomas, although the exact cellular origin may include radial glial cells, a precursor to ependymoma cells. Ependymomas are found throughout the CNS in the supratentorial, posterior fossa and spinal cord and they affect both pediatric and adult populations.

How are ependymomas classified?

Researchers classify ependymomas based on the degree of variability in a tumor size and shape  (pleomorphism), how fast the tumor cells are growing (mitotic count), crowding of the tumor cells (increased cellularity), the growth of tumor blood vessels (vascular proliferation), tendency for tumor cells to outgrow their blood supply (tumor necrosis), how much the tumor has spread into the surrounding normal tissue (invasion) and molecular and genetic features. The WHO classifies ependymomas into a low-grade variety named “ependymoma” and “myxopapillary ependymoma” (also called a “WHO grade II ependymoma”) and a more malignant type previously called “anaplastic ependymoma,” also classified as a “WHO grade III ependymoma”. There are also grade I ependymomas, including “subependymomas”. See our Pathology page for more information.

What age groups do ependymomas affect?

Although relatively uncommon, the disease can occur in any age group. Tumors of the posterior fossa (back part of the brain) are much more common in the pediatric population, and spinal cord tumors are more common in adults. The more malignant anaplastic ependymomas are more common in adults.

Is ependymoma cancer?

This question is nuanced and requires careful consideration. Generaly, central nervous system (CNS) tumors are considered malignant if they are grade III or IV. However, low grade tumors can recur and therefore should be evaluated carefully. Ependymoma tumors are typically designated as low grade (grade I or II) or high grade (grade III). Ependymoma in the brain or spine may cause problems and cause symptoms that require treatment, despite the size or grade. Like other CNS tumors, low and high grade ependymoma can recur. Grading is a better representation of the tumor growth, aggressiveness, and prognosis. It is important to talk with your medical provider about this important topic. You can find out more about the characteristics of each type by reviewing our Pathology section of our website.

What treatment is available for an individual diagnosed with an ependymoma?

Currently, the standard therapy for a low-grade ependymoma includes total surgery and may be followed by radiation therapy. Complete surgical resection is often not possible due to tumor location and concerns about damaging the surrounding brain during surgery. The situation is even more critical for patients with anaplastic ependymoma because of the higher growth rate and greater chance for tumor infiltration into the surrounding normal brain, preventing any possibility of complete tumor removal by surgery. In the case of incomplete resection or more aggressive grade, radiation therapy is often the prescribed therapy. For patients with the more aggressive anaplastic ependymoma, chemotherapy is often administered either before or after the radiation in the hope that infiltrating tumor cells will be eliminated. See our Treatment section for more details.

What happens if there is a recurrence?

Patients with recurrent tumors face a difficult challenge. Patients may undergo surgical re-resection of tumor. This often provides symptom improvement, reduces the tumor burden and confirms the presence of the tumor (as opposed to treatment-related necrosis) and the grade (II versus III). However, treatment for recurrent and anaplastic ependymomas relies on radiation therapy or the use of chemotherapy regimens. Conventional chemotherapy, also known as cytotoxic chemotherapy, has been a mainstay of cancer treatment for several decades. However, although there are reports that a variety of chemotherapy agents have been shown to have activity against ependymomas, there are no established standard regimens.

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