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Disseminated Intracranial and Spinal Dysembryoplastic Neuroepithelial Tumor: A Case Report with a Systematic Review
Maksymilian Niemczyk1, Justyna Fercho1,2,3,4, Oskar G Chasles1
1Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gdańsk, 80-210 Gdańsk, Poland.
Medical Sciences (Basel, Switzerland)
|May 27, 2026
Summary
Disseminated dysembryoplastic neuroepithelial tumors (DNETs) are rare, often presenting with spinal involvement and hydrocephalus. Multidisciplinary care and spinal MRI are crucial for managing these challenging intracranial and spinal DNETs.
Area of Science:
- Neuro-oncology
- Neurosurgery
- Pathology
Background:
- Dysembryoplastic neuroepithelial tumors (DNETs) are rare central nervous system neoplasms.
- Disseminated DNETs involving both intracranial and spinal compartments are exceptionally rare, with limited prior case reports.
Purpose of the Study:
- To present a rare case of disseminated DNETs involving the hippocampus and lumbar spine.
- To systematically review the existing literature on disseminated DNETs to identify key features and outcomes.
Main Methods:
- A case of a 47-year-old woman with surgically treated DNETs in the right hippocampus and lumbar spine.
- Systematic literature review of reported cases of disseminated DNETs (total n=6).
Main Results:
- Secondary lesions were predominantly spinal (83%).
- Obstructive hydrocephalus was common (67%), often requiring shunting, while epilepsy was rare (17%).
- MRI and surgical intervention (craniotomy/laminectomy) were primary diagnostic and treatment modalities.
Conclusions:
- Disseminated DNETs present significant management challenges.
- Consideration of spinal MRI in intracranial DNET cases and a multidisciplinary approach are recommended.
- Further molecular research and case registries are essential for understanding pathogenesis and optimizing treatment.