Neurologic Manifestations of Ependymoma Workup

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

Laboratory Studies

Laboratory studies are not helpful for diagnosis.

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Imaging Studies

  • MRI is the diagnostic tool of choice. It reveals discrete, heterogeneous masses with variable enhancement (see the images below). MRI, T2-weighted image, axial view, showing mixed 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, sagittalMRI, 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.
    • Evidence of calcification, necrosis, and cystic change are occasionally seen; hemorrhage is rare.
    • The tumor characteristically displaces rather than infiltrates brain parenchyma with minimal peritumoral edema.
  • CT scan is used in emergency situations, although its resolution is inferior to that of MRI. The CT appearance of ependymoma varies, but calcification is more evident on CT scan. 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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Procedures

  • Ventriculostomy is not required preoperatively because patients are usually stable. In fact, it should be avoided if possible because of the potentially fatal risk of upward herniation or hemorrhage within the tumor with brain stem compression.
  • Lumbar puncture is also contraindicated because of a similar risk of downward (tonsillar) herniation preoperatively.
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Histologic Findings

The presence of a classic, well-circumscribed lesion with moderate cellularity, punctuated by areas of an acellular, fibrillary zone (perivascular pseudorosettes) is common. Variants include the following:

  • Clear cell type with perinuclear halo
  • Papillary type with an extensive epithelial component
  • True rosette formation
  • Myxopapillary type with prominent perivascular and intercellular mucin
  • Rare melanotic type containing lipofuscin and no melanin pigment

Current World Health Organization (WHO) classification grades tumors as follows:

  • Subependymoma and myxopapillary tumors - Grade 1
  • Ependymoma - Grade 2
  • Anaplastic ependymoma - Grade 3

Only 10% of ependymomas metastasize to other areas of the neuraxis; these metastases are almost always associated with tumor recurrence at the primary site, which emphasizes the importance of local control.

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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].

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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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