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Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma
Article first published online: 10 SEP 2009
© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
Volume 20, Issue 3, pages 560–570, May 2010
How to Cite
Barton, V. N., Donson, A. M., Kleinschmidt-DeMasters, B. K., Birks, D. K., Handler, M. H. and Foreman, N. K. (2010), Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma. Brain Pathology, 20: 560–570. doi: 10.1111/j.1750-3639.2009.00333.x
- Issue published online: 12 APR 2010
- Article first published online: 10 SEP 2009
- Received 22 June 2009; accepted 26 August 2009.
- myxopapillary ependymoma;
- pediatric neuro-oncology
Myxopapillary ependymoma (MEPN) generally can be cured by gross total surgical resection and usually manifest a favorable prognosis. However, surgery is less curative in tumors that are large, multifocal or extend outside the thecal sac. Late recurrences may occur, particularly in pediatric patients. The role of adjuvant therapy is unclear in the clinical management of recurrent tumors. Clinical trial design requires a better understanding of tumor biology. Unique molecular features of MEPN were investigated by using microarray technology to compare the gene expression of five pediatric MEPN to 24 pediatric intracranial ependymoma (EPN). The upregulation of three genes of interest, homeobox B13 (HOXB13), neurofilament, light polypeptide (NEFL) and PDGFRα, was further studied by immunohistochemistry in a larger cohort that included adult MEPN and EPN specimens. Protein expression in MEPN was compared to subependymoma, spinal EPN, intracranial EPN and normal fetal and adult ependyma. Immunoreactivity for HOXB13, NEFL and PDGFRα was strongest in MEPN and virtually absent in subependymoma. Spinal and intracranial EPN generally expressed weak or focal staining. MEPN manifests unique gene and protein expression patterns compared to other EPNs. Aberrant expression of HOXB13 suggests possible recapitulation of developmental pathways in MEPN tumorigenesis. PDGFRα may be a potential therapeutic target in recurrent MEPN.