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Ependymoma is a tumor that arises from the ependyma, a tissue of the
central nervous system. Usually, in children the location is
intracranial, while in adults it is spinal. The common location of
intracranial ependymoma is the fourth ventricle. Rarely, ependymoma
can occur in the pelvic cavity.
Syringomyelia can be caused by an ependymona. Ependymomas are also
seen with Neurofibromatosis Type II.
Contents [hide]
1 Morphology
2 Ependymoma tumors
3 References
4 External links
4.1 Images and technical information
[edit] Morphology
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.[1]
[edit] Ependymoma tumors
Ependymomas make up about 5% of adult intracranial gliomas and up to
10% of childhood tumors of the central nervous system (CNS). Their
occurrence seems to peak at age 5 years and then again at age 34.
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 headache, nausea
and vomiting by obstructing the flow of cerebrospinal fluid. This
obstruction may also cause hydrocephalus.
About 85% of ependymomas are benign myxopapillary ependymoma (MPE).
MPE is a localized and slowly growing, low-grade tumor. Although some
ependymomas are of a more anaplastic and malignant type, most of them
are not anaplastic. For this reason, well-differentiated ependymomas
are usually treated with radiation therapy only. For other
ependymomas, total surgical removal is the preferred treatment and
those that cannot be totally removed also require radiation therapy.
The malignant (anaplastic) varieties of this tumor, malignant
ependymoma and the ependymoblastoma, are treated similarly to
medulloblastoma but the prognosis is much less favorable. Malignant
ependymomas may be treated with a combination of radiation therapy
and chemotherapy. Ependymoblastomas, which occur in infants and
children younger than 5 years of age, may spread through the
cerebrospinal fluid and usually require radiation therapy. 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. It usually affects people over 40 years of age
and more often affects men than women. The subependymal giant-cell
astrocytoma, also called giant-cell glioma, is typically associated
with tuberous sclerosis but can occur independent of that condition.
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[2] or may be confused with a
sacrococcygeal teratoma.[3]
[edit] References
^ Kumar, et al. (2005). The Central Nervous System. Pathologic Basis
of Disease. 7th Edition. Philadelphia: Elsevier Saunders.
^ Aktug T, Hakgüder G, Sarioglu S, Akgür FM, Olguner M,
Pabuçcuoglu U. (2000) Sacrococcygeal extraspinal ependymomas:
the role of coccygectomy. J Pediatr Surg. 35(3):515-518. PubMed
^ 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): 43741. PMID 9783311.
[edit] External links
[edit] Images and technical information
CINN
Illustration
Ependymoma Topics - MedPix
Ependymoma Information from ABTA (PDF) for Completely Resected,
Differentiated, Supratentorial Ependymoma]
St. Jude Ependymoma Information
Cancer Back Up - Ependymoma
Chronic Back Pain: Clinical Quiz [1]
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