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Neurologic Manifestations of Ependymoma Treatment & Management

  • Author: Subrata Ghosh, MD, MBBS, MS; Chief Editor: Stephen A Berman, MD, PhD, MBA  more...
 
Updated: Mar 19, 2015
 

Medical Care

A multimodality approach that encompasses maximal surgical resection in combination with adjuvant therapy is critical for achieving optimal disease control.[2] Preoperative and perioperative steroids are recommended to help limit edema and alleviate some symptoms.

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Surgical Care

Surgery remains the most effective therapy for this tumor. It establishes tissue diagnosis, restores normal cerebrospinal fluid flow, and can be used to attempt total removal of the tumor.

A second-look surgery for unexpected residual lesions that are seen on postoperative imaging in an operable location is encouraged in patients with noninvasive, benign histology.

Permanent cerebrospinal fluid (CSF) diversion with ventriculoperitoneal shunt is rarely required.

Postoperative radiation therapy substantially improves survival. Although not proven, some dose-to-response relationship probably exists.

Newer methods that target only the local tumor bed, such as high fractionation radiotherapy or stereotactic radiosurgery, may permit potential dose reduction as compared with conventional radiotherapy. It provides effective tumor control, which may help limit complications such as cognitive dysfunction, growth delay, and hypothyroidism.

Craniospinal axis radiation is recommended only for patients with radiological or pathological evidence of spinal seeding.

Overall, results of chemotherapy are disappointing.[3] Multidrug combinations using VP-16 etoposide, vincristine, CCNU (lomustine), and cisplatin offer limited benefit in patients with recurrent disease.

Gross total resection (GTR) is associated with the lowest rates of mortality, the best overall survival, and the longest progression-free survival rates. Patients with WHO grade II tumors had better overall survival after GTR plus external-beam radiation therapy (EBRT) and better progression-free survival rates than after GTR alone. Patients with WHO grade III tumors had better overall survival after subtotal resection plus EBRT.[4]

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Consultations

See the list below:

  • Neurosurgeon
  • Neurologist
  • Radiation oncologist
  • Medical oncologist
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Contributor Information and Disclosures
Author

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

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, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian Medical Protective Association, Neurocritical Care Society, Canadian Critical Care Society, Canadian Critical Care Society, Canadian Neurocritical Care Society, Canadian Neurological Sciences Federation

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

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, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

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, Society for Neuroscience

Disclosure: Nothing to disclose.

References
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  2. Kim JH, Huang Y, Griffin AS, Rajappa P, Greenfield JP. Ependymoma in children: molecular considerations and therapeutic insights. Clin Trans Oncol. April 2013. [Medline].

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

  4. Cage TA, Clark AJ, Aranda D, et al. A systematic review of treatment outcomes in pediatric patients with intracranial ependymomas. J Neurosurg Pediatr. 2013 Jun. 11(6):673-81. [Medline].

  5. Dützmann S, Schatlo B, Lobrinus A, et al. A multi-center retrospective analysis of treatment effects and quality of life in adult patients with cranial ependymomas. J Neurooncol. 2013 Jun 29. [Medline].

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

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

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

  10. Graham DI, Lantos PL, eds. Greenfield's Neuropathology. 6th ed. Arnold Press; 1997. 636-44.

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

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

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

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

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

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

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

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

  19. Chamberlain MC, Chang E, Maravilla KR. Lumbar spine neuroarthropathy (Charcot joint) caused by a myxopapillary ependymoma. J Neurooncol. 2014 Apr. 117(2):375-6. [Medline].

  20. Wang H, Zhang S, Rehman SK, Zhang Z, Li W, Makki MS, et al. Clinicopathological features of myxopapillary ependymoma. J Clin Neurosci. 2014 Apr. 21(4):569-73. [Medline].

 
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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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