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World Neurosurg. 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.

Author information

1
College of Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
2
Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
3
Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
4
Department of Neurological Surgery, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA. Electronic address: brad.elder@osumc.edu.

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

PMID:
27671879
PMCID:
PMC5550327
DOI:
10.1016/j.wneu.2016.09.063
[Indexed for MEDLINE]
Free PMC Article

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