Ependymoma
Ependymoma | |
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Micrograph of an ependymoma. H&E stain. | |
Specialty | Neuro-oncology |
Prognosis | Five-year survival rate: 83.9%[1] |
Frequency | 200 new cases each year in the United States[2] |
An ependymoma is a
Syringomyelia can be caused by an ependymoma. Ependymomas are also seen with neurofibromatosis type II.
Signs and symptoms
Source:[3]
- severe headache
- visual loss (due to papilledema)
- vomiting
- bilateral Babinski sign
- drowsiness (after several hours of the above symptoms)
- gait change (rotation of feet when walking)
- impaction/constipation
- back flexibility
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
It has been suggested that ependymomas are derived from radial glia, despite their name suggesting an ependymal origin.[5]
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Micrograph of a myxopapillary ependymoma. HPS stain.
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Ependymoma of 4.ventricle in MRI.
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Ependymoma of 4.ventricle in MRI.
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Ependymoma of 4.ventricle in MRI. Left without, right with contrast-enhancement.
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 35. 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. They may also arise in the spinal cord, conus medullaris and supratentorial locations.[6] Other symptoms can include (but are not limited to): loss of appetite, difficulty sleeping, temporary inability to distinguish colors, uncontrollable twitching, seeing vertical or horizontal lines when in bright light, and temporary memory loss. It should be remembered that these symptoms also are prevalent in many other illnesses not associated with ependymoma.[citation needed]
About 10% of ependymomas are
Although some ependymomas are of a more
Extraspinal ependymoma (EEP), also known as extradural ependymoma, may be an unusual form of teratoma[10] or may be confused with a sacrococcygeal teratoma.[11]
Treatment
Guidelines for initial management for ependymoma are maximum surgical resection followed by radiation.[12] Chemotherapy is of limited use and reserved for special cases including young children and those with tumor present after resection. Prophylactic craniospinal irradiation is of variable use and is a source of controversy given that most recurrence occurs at the site of resection and therefore is of debatable efficacy.[13][12] Confirmation of cerebrospinal infiltration warrants more expansive radiation fields.[14]
Prognosis of recurrence is poor and often indicates palliative care to manage symptoms.[15]
References
- ^ "Ependymoma Diagnosis and Treatment". National Cancer Institute. Retrieved March 8, 2023.
- ^ "Ependymoma". St. Jude Children's Research Hospital. Retrieved March 8, 2023.
- ^ PRITE 2010 Part II q.13
- ISBN 0-7216-0187-1.[page needed]
- PMID 17179988.
- ^ Goel A, Gaillard F. "Ependymoma". Radiopaedia.org. Retrieved 12 September 2014.
- ^ .
- PMID 22907705.
- ^ Pan E, Prados MD (2003). "Ependymoma". Holland-Frei Cancer Medicine (6th ed.).
- PMID 10726703.
- PMID 9783311.
- ^ PMID 14732890.
- S2CID 37438304.
- ^ "Ependymoma". The Lecturio Medical Concept Library. Retrieved 19 July 2021.
- PMID 18406885.