Ependymoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Pathophysiology

• Ependymomas represent a relatively broad group of glial tumours which share common origin from differentiated ependymal cells lining the ventricles of the brain or the central canal of the spinal cord. They account for ~5% of all neuroepithelial neoplasms, ~10% of all paediatric brain tumours and up to 33% of brain tumours occurring in those less than 3 years of age.
• Ependymomas can occur anywhere, but certain location are typical. Common locations include:

• Floor of the fourth ventricle (common location in children)
• Spinal cord ependymoma
• Myxopapillary ependymoma (conus medullaris)
• Supratentorial ependymoma

• The subependymomas, variant of the ependymoma, arise in the fourth ventricle but may occur in the septum pellucidum and the cervical spinal cord.

Gross Pathology

• Ependymomas are well-encapsulated tumors and usually arise from the floor of the fourth ventricle, situated in the lower back portion of the brain.

Microscopic Pathology

Ependymomas are composed of cells with regular, round to oval nuclei. There is a variably dense fibrillary background. Tumor cells may form gland-like round or elongated structures that resemble the embryologic ependymal canal, with long, delicate processes extending into the lumen; more frequently present are perivascular pseudorosettes in which tumor cells are arranged around vessels with an intervening zone consisting of thin ependymal processes directed toward the wall of the vessel.. Ependymal rosettes are rare but pathognomonic feature.

Micrograph of an ependymoma

True ependymal rosette consisting of tumor cells arranged around well-defined lumens forming gland-like structures.

Associated Conditions

The subependymal giant-cell astrocytoma, also called giant-cell glioma, is typically associated with tuberous sclerosis but can occur independent of that condition.