Neurologic Manifestations of Ependymoma 

  • Author: Subrata Ghosh, MD, MBBS; Chief Editor: Stephen A Berman, MD, PhD, MBA   more...
 
Updated: Aug 29, 2011
 

Background

Ependymomas are neoplasms of ependymal cells that occur throughout the entire neuraxis in association with the lining of the cerebral ventricles and central canal of the spinal cord.

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Pathophysiology

Ependymomas occur most commonly in the intracranial and intraspinal areas, with lesions rarely occurring in the sacral area. Other unusual ectopic sites of ependymoma are the mediastinum, ovary, and broad ligament. In general, the anatomic location determines the pathophysiological manifestations of the tumor. Supratentorial tumors present with mass effect, focal neurological signs, and occasional obstruction of ventricular outflow. The relationship with the ventricular system is more apparent in tumors of the posterior fossa (mostly of the fourth ventricle), which usually present with obstructive hydrocephalus with or without signs of brain stem compression. See the images below.

CT scan without contrast, axial view, demonstratesCT scan without contrast, axial view, demonstrates mixed but predominantly hyperdense tumor in the posterior fossa with severe obstructive hydrocephalus. CT scan with contrast, axial view shows moderatelyCT scan with contrast, axial view shows moderately intense contrast enhancement (compare with the previous image).
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Epidemiology

Frequency

United States

Ependymomas are infrequent tumors, representing 2-8% of all brain tumors. However, ependymomas are the third most common brain tumor in children (8-12%) with up to 30% occurring in children younger than 3 years. Half of the ependymomas occur in the first 2 decades of life; two-thirds are located in the posterior fossa (>90% are in the fourth ventricle). Interestingly, despite their overall low frequency, ependymomas are the most frequent neuroepithelial tumors of the spinal cord.

Mortality/Morbidity

From the biological perspective, ependymomas do not usually proliferate rapidly, are not invasive, and usually do not metastasize.[1] The associated morbidity can mainly be accounted for by the local space-occupying effects of the tumor. In unusual cases, the risk of sudden death from large intracranial ependymomas results from increased intracranial pressure secondary to obstructive hydrocephalus.

Race

No race predilection is reported.

Sex

No sex predilection is reported.

Age

Peak age at presentation ranges from 7 weeks to 16 years with a mean of 3.7 years. A second, lower peak age of presentation occurs in the third decade of life.

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Contributor Information and Disclosures
Author

Subrata Ghosh, MD, MBBS  Staff Physician, Division of Neurosurgery, St. Luke's Episcopal Hospital, Texas Medical Center, Houston; Assistant Professor of Neurosurgery, Baylor College of Medicine

Subrata Ghosh, MD, MBBS is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, Congress of Neurological Surgeons, and Texas Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Draga Jichici, MD, FRCP, FAHA  Associate Clinical Professor, Department of Neurology and Critical Care Medicine, McMaster University School of Medicine, Canada

Draga Jichici, MD, FRCP, FAHA is a member of the following medical societies: American Academy of Neurology, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Congress of Neurological Sciences, Canadian Critical Care Society, Canadian Medical Protective Association, Canadian Neurocritical Care Society, Neurocritical Care Society, Royal College of Physicians and Surgeons of Canada, and Society of Critical Care Medicine (USA)

Disclosure: Nothing to disclose.

Specialty Editor Board

Rodrigo O Kuljis, MD  Esther Lichtenstein Professor of Psychiatry and Neurology, Director, Division of Cognitive and Behavioral Neurology, Department of Neurology, University of Miami School of Medicine

Rodrigo O Kuljis, MD is a member of the following medical societies: American Academy of Neurology and Society for Neuroscience

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Jorge C Kattah, MD  Head, Associate Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Chief Editor

Stephen A Berman, MD, PhD, MBA  Professor of Neurology, University of Central Florida College of Medicine

Stephen A Berman, MD, PhD, MBA is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

References

  1. Nazar GB, Hoffman HJ, Becker LE, et al. Infratentorial ependymomas in childhood: prognostic factors and treatment. J Neurosurg. Mar 1990;72(3):408-17. [Medline].

  2. Moynihan TJ. Ependymal tumors. Curr Treat Options Oncol. Dec 2003;4(6):517-23. [Medline].

  3. [Best Evidence] Massimino M, Buttarelli FR, Antonelli M, Gandola L, Modena P, Giangaspero F. Intracranial ependymoma: factors affecting outcome. Future Oncology. March 2009;5(2):207-16. [Medline]. [Full Text].

  4. Bhattacharjee MB, Armstrong DD, Vogel H, Cooley LD. Cytogenetic analysis of 120 primary pediatric brain tumors and literature review. Cancer Genet Cytogenet. Aug 1997;97(1):39-53. [Medline].

  5. Bigner SH, McLendon RE, Fuchs H, et al. Chromosomal characteristics of childhood brain tumors. Cancer Genet Cytogenet. Sep 1997;97(2):125-34. [Medline].

  6. Ernestus RI, Schroder R, Stutzer H, Klug N. The clinical and prognostic relevance of grading in intracranial ependymomas. Br J Neurosurg. Oct 1997;11(5):421-8. [Medline].

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  8. Kaye AH, Laws E Jr, eds. Brain Tumors: An Encyclopedic Approach. First ed. Churchill Livingstone; 1997:493-504.

  9. Kleihues P et al. Pathology & Genetics. Tumors of the Nervous System. International Agency for Research on Cancer (IARC)/World Health Organization. 1997;96-109.

  10. Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumours. Brain Pathol. Jul 1993;3(3):255-68. [Medline].

  11. Kun LE. Brain tumors. Challenges and directions. Pediatr Clin North Am. Aug 1997;44(4):907-17. [Medline].

  12. McLaughlin MP, Marcus RB, Buatti JM, et al. Ependymoma: results, prognostic factors and treatment recommendations. Int J Radiat Oncol Biol Phys. Mar 1 1998;40(4):845-50. [Medline].

  13. Osborn AG. Diagnostic Neuroradiology: A Text and Atlas. First ed. Mosby; 1994:566-70.

  14. Russell DS, et al. Pathology of Tumors of the Nervous System. 4th ed. Arnold Press; 1977:203-26.

 
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CT scan without contrast, axial view, demonstrates mixed but predominantly hyperdense tumor in the posterior fossa with severe obstructive hydrocephalus.
CT scan with contrast, axial view shows moderately intense contrast enhancement (compare with the previous image).
MRI, T2-weighted image, axial view, showing mixed (isodensity and hyperdensity) heterogenous nature of the tumor with some peritumoral edema.
MRI, T1-weighted image, without contrast, sagittal view, showing the posterior fossa location, mixed (hypodensity and isodensity) signal intensity and tending to grow out of the fourth ventricle.
 
 
 
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