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Pediatric septal dysembryoplastic neuroepithelial tumor (sDNT): case-based update
Ranbir Ahluwalia1,2, Lili Miles3, Laura Hayes4
1Florida State College of Medicine, 250 E Colonial Dr #200, Orlando, FL, 32801, USA. ra16@med.fsu.edu.
Summary
This study reviewed a case of septal dysembryoplastic neuroepithelial tumor (sDNT), finding endoscopic biopsy effective for diagnosis and treatment. This rare pediatric condition often presents with epilepsy and may respond to PDGFRA-targeted therapies.
Area of Science:
- Neuro-oncology
- Pediatric Neurosurgery
- Molecular Pathology
Background:
- Septal dysembryoplastic neuroepithelial tumor (sDNT) is a rare pediatric neoplasm.
- sDNT commonly manifests as intractable epilepsy or headaches.
- sDNT is a distinct entity from dysembryoplastic neuroepithelial tumor (DNT).
Observation:
- A case of sDNT was reviewed and compared with seven previously reported cases.
- Magnetic Resonance Imaging (MRI) typically reveals a lobular interventricular mass involving the septum.
- Approximately 80% of sDNT cases exhibit mutations in platelet-derived growth factor receptor A (PDGFRA).
Findings:
- Gross total resection is the primary treatment for sDNT, often leading to complete clinical resolution.
- Endoscopic biopsy is validated as a diagnostic and therapeutic intervention for sDNT.
- PDGFRA mutations suggest potential for targeted molecular therapies in sDNT treatment.
Implications:
- Endoscopic biopsy offers a minimally invasive approach for sDNT diagnosis and management.
- Understanding sDNT's distinct genotype, particularly PDGFRA mutations, opens avenues for novel therapeutic strategies.
- This review contributes to the literature on sDNT, enhancing diagnostic and treatment paradigms for this rare pediatric tumor.
