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

Gross Pathology

• Ependymomas develop from cells that line the brain ventricles and spinal canal.
• They are well-encapsulated tumors and usually arise from the floor of the fourth ventricle, situated in the lower back portion of the brain.
• The subependymomas, variant of the ependymoma, arise in the fourth ventricle but may occur in the septum pellucidum and the cervical spinal cord.

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.