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J Neuropathol Exp Neurol. 2016 Apr;75(4):358-65. doi: 10.1093/jnen/nlw007. Epub 2016 Feb 27.

Malignant Transformation of a Dysembryoplastic Neuroepithelial Tumor (DNET) Characterized by Genome-Wide Methylation Analysis.

Author information

1
From the Department of Neurosurgery (DHH, WM, PF, JG, AW), Department of Pathology, Institute of Neuropathology (OS), Department of Neurosurgery, Epilepsy Center (MH), and Department of Neuroradiology (HU), Medical Center - University of Freiburg, Freiburg, Germany; and Department of Neuropathology, University Heidelberg, Im Neuenheimer Feld 224 and German Cancer Consortium (DKTK), CCU Neuropathology, German Cancer Research Center (DKFZ), Heidelberg, Germany (DC, DS).
2
From the Department of Neurosurgery (DHH, WM, PF, JG, AW), Department of Pathology, Institute of Neuropathology (OS), Department of Neurosurgery, Epilepsy Center (MH), and Department of Neuroradiology (HU), Medical Center - University of Freiburg, Freiburg, Germany; and Department of Neuropathology, University Heidelberg, Im Neuenheimer Feld 224 and German Cancer Consortium (DKTK), CCU Neuropathology, German Cancer Research Center (DKFZ), Heidelberg, Germany (DC, DS). astrid.weyerbrock@uniklinik-freiburg.de.

Abstract

Dysembryoplastic neuroepithelial tumors (DNET) are considered to be rare, benign, and associated with chronic epilepsy. We present the case of a 28-year-old man with a history of epilepsy since age 12. Surgery of an occipital cortical lesion in 2009 revealed a DNET. Five years later, a recurrent tumor at the edge of the resection cavity was removed, and the tissue underwent an intensive diagnostic workup. The first tumor was unequivocally characterized as a DNET, but neuropathological diagnostics of the recurrent tumor revealed a glioblastoma. After 6 months, another recurrent tumor was detected next to the location of the original tumor, and this was also resected. An Illumina 450 K beadchip methylation array was performed to characterize all of the tumors. The methylation profile of these tumors significantly differed from other glioblastoma and epilepsy-associated tumor profiles and revealed a DNET-like methylation profile. Thus, molecular characterization of these recurrent tumors suggests malignant transformation of a previously benign DNET. We found increased copy number changes in the recurrent DNET tumors after malignant transformation. Modern high-throughput analysis adds essential molecular information in addition to standard histopathology for proper identification of rare brain tumors that present with an unusual clinical course.

KEYWORDS:

Copy number changes; Dysembryoplastic neuroepithelial tumor (DNET); Epigenetic alteration; Illumina 450 K beadchip methylation array; Malignant DNET; Methylation.

PMID:
26921879
DOI:
10.1093/jnen/nlw007
[Indexed for MEDLINE]

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