Ependymoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Pathophysiology

Gross Pathology

They develop from cells that line both the hollow cavities of the brain and the canal containing the spinal cord, but they usually arise from the floor of the fourth ventricle, situated in the lower back portion of the brain, where they may produce obstruction of the flow of cerebrospinal fluid. This obstruction may also cause hydrocephalus.

The subependymoma, a variant of the ependymoma, is apt to arise in the fourth ventricle but may occur in the septum pellucidum and the cervical spinal cord. Arising in the walls of the lateral ventricles over the basal ganglia, this tumor tends to cause obstruction when it becomes large. It is a well-encapsulated tumor, however, and generally has a very benign course.

Extraspinal ependymoma (EEP), also known as extradural ependymoma, may be an unusual form of teratoma[1] or may be confused with a sacrococcygeal teratoma.[2]

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.[3]

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.

References

  1. Aktuğ T, Hakgüder G, Sarioğlu S, Akgür FM, Olguner M, Pabuçcuoğlu U. (2000) Sacrococcygeal extraspinal ependymomas: the role of coccygectomy. J Pediatr Surg. 35(3):515-518. PubMed
  2. Hany MA, Bouvier R, Ranchère D, Bergeron C, Schell M, Chappuis JP, Philip T, Frappaz D (1998). "Case report: a preterm infant with an extradural myxopapillary ependymoma component of a teratoma and high levels of alpha-fetoprotein.". Pediatric hematology and oncology 15 (5): 437–41. PMID 9783311.
  3. Kumar, et al. (2005). The Central Nervous System. Pathologic Basis of Disease. 7th Edition. Philadelphia: Elsevier Saunders.

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