Journal
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY
Volume 70, Issue 10, Pages 859-878Publisher
OXFORD UNIV PRESS INC
DOI: 10.1097/NEN.0b013e3182302475
Keywords
Dysembryoplastic neuroepithelial tumor; Epilepsy; Molecular genetics; Outcome
Categories
Funding
- Medical Research Council [0600934]
- University College London Hospital Comprehensive Biomedical Centre [CBRC31]
- Samantha Dickson Brain tumor trust [SDBTT0805]
- Department of Health's National Institute for Health Research Biomedical Research Centres at University College London/Hospital
- Medical Research Council [G0600934] Funding Source: researchfish
- MRC [G0600934] Funding Source: UKRI
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Simple and complex forms of dysembryoplastic neuroepithelial tumors (DNTs) are readily recognizable but forms with diffuse growth pattern, and hybrid tumors, that is, mixed DNT and ganglioglioma (DNT/GG), are more contentious entities. Rare DNTs have shown aggressive behavior. We reviewed cortical growth patterns, immunophenotype (including CD34, nestin and calbindin), genetic profile, and outcome in 101 DNT in adults. Simple (n = 18), complex (n = 31), diffuse (n = 35) DNT, and mixed DNT/GG (n = 17) showed no difference in age of onset, associated seizure type, or outcome (67.5% free from seizure; mean follow-up, 6 years). CD34 was seen in 61%, calbindin in 57%, and nestin in 86% of all DNT types; these markers were less common in simple DNT. Peritumoral cortical changes (Layer I hypercellularity [61%], satellite nodules [51.6%]) were frequent, but dyslamination (cortical dysplasia) was not identified. Molecular genetic abnormalities identified in 17 cases were IDH1 mutation (n - 3), 1p/19q loss (n - 10), isolated loss 9q (n - 2), and PTEN loss (n - 3), which were not associated with tumor type or location, higher cell proliferation, or distinguishing clinical features (mean age of epilepsy onset, 9 years; age at surgery = 31 years; 69% free from seizure); none had progression on magnetic resonance imaging (mean follow-up, 6 years). No single feature was predictive of seizure-free outcome, but there was a trend for better outcome in CD34-positive tumors (p = 0.07). One case has shown transformation to a higher grade. This study supports the existence of a range of sub-types of DNT some with overlapping features with ganglioglioma; molecular genetic abnormalities were not predictive of atypical behavior.
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