Disseminated Intracranial and Spinal Dysembryoplastic Neuroepithelial Tumor: A Case Report with a Systematic Review

Background: Disseminated intracranial and spinal dysembryoplastic neuroepithelial tumors (DNETs) are exceptionally rare, with only five prior cases reported. Methods: This study presents a case of a 47-year-old woman with DNETs in the right hippocampus and lumbar spine, treated surgically, and systematically reviews the existing literature (total n = 6). Results: While primary tumors occurred in both locations, secondary lesions were predominantly spinal (83%). The key features included frequent obstructive hydrocephalus (67%, often requiring shunting) but rare epilepsy (n = 1, 17%). Two fatal outcomes were possibly associated with West Nile virus infection (the S100B protein pathway). MRI was performed in all cases, and surgical intervention (craniotomy/laminectomy) constituted the primary treatment modality (67%). Conclusions: This multifocal presentation poses significant challenges, underscoring the potential need for spinal MRI in intracranial DNET cases and multidisciplinary management. Further molecular studies and case registries are crucial to understand pathogenesis and optimize care.