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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Myxopapillary ependymoma


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Overview

What is myxopapillary ependymoma?

How might myxopapillary ependymoma be treated?

What is myxopapillary ependymoma?

Myxopapillary ependymoma (MEPN) is a slow-growing ependymoma (a type of glioma, which is a tumor that arises from the supportive tissue of the brain).[1] They tend to occur in the lower part of the spinal column and are usually considered to be benign, low-grade or grade I tumors.[2] The age of diagnosis ranges from 6 to 82 years. Symptoms of an ependymoma are related to the location and size of the tumor and may include nausea; vomiting; headache; pain; numbness; bowel or bladder symptoms; and various other signs and symptoms. The cause of ependymomas is unknown.[2] They are known to recur locally (more commonly in individuals diagnosed in childhood).[3][1] Treatment may vary depending on the location, grade, and whether the tumor has spread to the spine,[2] but typically includes aggressive surgery;[1] management may also include chemotherapy and radiation therapy.[3]
Last updated: 4/7/2011

How might myxopapillary ependymoma be treated?

Standard treatment of myxopapillary ependymoma is aggressive surgery. It can generally can be cured by gross total surgical resection, and there is usually a favorable prognosis. However, surgery is typically less curative in tumors that are large, multifocal or extend outside the thecal sac. Late recurrences may occur, particularly in pediatric individuals.[1] Treatment strategies may also include radiation, but the use of chemotherapy remains more controversial; chemotherapy has been widely used in pediatric individuals due to more aggressive disease in these individuals.[4] The role of adjuvant chemotherapy or radiotherapy is unclear for the subset of individuals with recurrence or in individuals in whom gross total resection cannot be achieved.[1]
Last updated: 6/8/2011

References
  1. Valerie N Barton et al. Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma. Brain Pathology. May 2010; 20(3):560-570. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2871180/?tool=pubmed. Accessed 4/7/2011.
  2. Ependymoma. American Brain Tumor Association. http://www.abta.org/siteFiles/pdflibrary/ABTA_Ependymoma_Pamph_7%202%2010.pdf. Accessed 4/7/2011.
  3. Jeffrey N Bruce. Ependymoma. Medscape. January 26, 2009; http://www.medscape.com/viewarticle/465375_2. Accessed 4/7/2011.
  4. Shonka NA. Targets for therapy in ependymoma. Targeted Oncology. March 29, 2011; Epub:http://www.ncbi.nlm.nih.gov/pubmed/21445635. Accessed 4/7/2011.


See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.