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

  • Case-Based Review
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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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Authors and Affiliations

  1. Florida State College of Medicine, 250 E Colonial Dr #200, Orlando, FL, 32801, USA

    Ranbir Ahluwalia

  2. Florida State University College of Medicine, 1115 W Call St., Tallahassee, FL, 32304, USA

    Ranbir Ahluwalia

  3. Department of Pathology, Nemours Children’s Hospital, Orlando, FL, USA

    Lili Miles

  4. Department of Neuroradiology, Nemours Children’s Hospital, Orlando, FL, USA

    Laura Hayes

  5. Department of Neurological Surgery, Nemours Children’s Hospital, Orlando, FL, USA

    Andrea Scherer

Authors
  1. Ranbir Ahluwalia
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  2. Lili Miles
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  3. Laura Hayes
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  4. Andrea Scherer
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Correspondence to Ranbir Ahluwalia.

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The other authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper. The authors have no personal or institutional financial interest in drugs, materials, or devices described in their submissions.

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Ahluwalia, R., Miles, L., Hayes, L. et al. Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update. Childs Nerv Syst 36, 1127–1130 (2020). https://doi.org/10.1007/s00381-020-04581-y

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  • DOI: https://doi.org/10.1007/s00381-020-04581-y

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