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Child's Nervous System
Published in: Child's Nervous System 6/2020

01-06-2020 | Hydrocephalus | Case-Based Review

Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update

Authors: Ranbir Ahluwalia, Lili Miles, Laura Hayes, Andrea Scherer

Published in: Child's Nervous System | Issue 6/2020

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Abstract

Purpose

The purpose of this study was to review a case of a septal dysembryoplastic neuroepithelial tumor (sDNT) and compare it to cases reported in the current literature.

Methods

We review a case of sDNT and compare with 7 other previously noted cases in the literature.

Results

The mainstay treatment is gross total resection, and most patients achieve full clinical resolution. Septal dysembryoplastic neuroepithelial tumor (sDNT) is a rare pediatric disease most commonly presenting as intractable epilepsy or headache. sDNT has been recognized as a genotypically distinct entity from DNT. A high frequency (~ 80%) of mutations of platelet-derived growth factor receptor A (PDGFRA) has been isolated in sDNT and could form the basis for future therapy. MRI is most commonly used to radiographically diagnose the disease and usually demonstrates a lobular interventricular mass involving the septum, potentially extending to the third ventricle.

Conclusions

Our case and literature review validates endoscopic biopsy as a diagnostic and therapeutic intervention.
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Metadata
Title
Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update
Authors
Ranbir Ahluwalia
Lili Miles
Laura Hayes
Andrea Scherer
Publication date
01-06-2020
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 6/2020
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-020-04581-y

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