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Case Reports
. 2016 Dec:96:608.e1-608.e4.
doi: 10.1016/j.wneu.2016.09.063. Epub 2016 Sep 23.

Genetic Characterization of a Multifocal Ganglioglioma Originating Within the Spinal Cord

Joshua L Wang  1 , Christopher S Hong  1 , Jose Otero  2 , Vinay K Puduvalli  3 , J Bradley Elder  4
Affiliations
Case Reports

Genetic Characterization of a Multifocal Ganglioglioma Originating Within the Spinal Cord

Joshua L Wang et al. World Neurosurg. 2016 Dec.

Abstract

Background: Gangliogliomas are rare, low-grade intra-axial tumors that exhibit both neuronal and glial components. Although the vast majority present as an intracranial lesion, a rare subset exist as isolated lesions of the spinal cord. Gangliogliomas have also been shown to harbor mutations in the p53 tumor suppressor gene and BRAF oncogene. Previous studies in ganglioglioma have correlated p53 mutations with histologic transformation and BRAF mutations with worse prognosis.

Case description: In this report, we describe a 35-year-old female who presented with multifocal ganglioglioma, involving both the conus medullaris and filum terminale. The dominant lesion in the filum terminale was resected, which revealed World Health Organization I grade, p53 mutant, and BRAF wildtype status. Our study documents the first report of a multifocal ganglioglioma, originating within the spinal cord.

Conclusions: Importantly, this case contradicts previous reports of p53 and BRAF mutations portending worsened tumor behavior and prognosis and demonstrates that further studies are needed to delineate the role of genetic characterization in the biologic understanding and management of gangliogliomas.

Keywords: BRAF; Ganglioglioma; Multifocal ganglioglioma; p53.

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Conflict of interest statement

Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
(A) Sagittal view of T1-weighted lumbar spine magnetic resonance imaging (MRI) after contrast administration demonstrating 2 dominant, homogenously enhancing masses within the spinal canal (arrows). Other masses not shown. (B) An axial slice from the same T1-weighted postcontrast MRI redemonstrates the enhancing mass within the filum terminale (arrow). (C) Sagittal view of a T1-weighted lumbar spine MRI after contrast administration taken 6 months after surgery demonstrating postoperative changes (arrow) and no evident changes in the remaining lesions.
Figure 2
Figure 2
Representative low-power (20× magnification, top row) and high-power (40× magnification, bottom row) views of the resected tumor showing characteristics of World Health Organization grade I ganglioglioma, stained for (A) hematoxylin-eosin, (B) Ki-67, and (C) p53 immunohistochemistry.
See this image and copyright information in PMC

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