Unique Molecular Characteristics of Pediatric Myxopapillary Ependymoma

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...

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Published in:Brain pathology (Zurich, Switzerland) Switzerland), 2010-05, Vol.20 (3), p.560-570
Main Authors: Barton, Valerie N., Donson, Andrew M., Kleinschmidt-DeMasters, Bette K., Birks, Diane K., Handler, Michael H., Foreman, Nicholas K.
Format: Article
Language:English
Subjects:
Adolescent
Adult
Child
Cohort Studies
Ependymoma - genetics
Ependymoma - metabolism
Ependymoma - physiopathology
Female
HOX
Humans
Male
microarray
myxopapillary ependymoma
NEFL
Nerve Tissue Proteins - analysis
Nerve Tissue Proteins - genetics
Nerve Tissue Proteins - metabolism
PDGFRα
pediatric neuro-oncology
Retrospective Studies
Spinal Cord Neoplasms - genetics
Spinal Cord Neoplasms - metabolism
Spinal Cord Neoplasms - physiopathology
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Summary: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.
ISSN:1015-6305
1750-3639
DOI:10.1111/j.1750-3639.2009.00333.x