{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T14:17:41Z","timestamp":1778681861625,"version":"3.51.4"},"reference-count":19,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T00:00:00Z","timestamp":1778630400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Medical Sciences"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/medsci14020250","type":"journal-article","created":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T12:46:55Z","timestamp":1778676415000},"page":"250","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Disseminated Intracranial and Spinal Dysembryoplastic Neuroepithelial Tumor: A Case Report with a Systematic Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-7959-7942","authenticated-orcid":false,"given":"Maksymilian","family":"Niemczyk","sequence":"first","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9845-4961","authenticated-orcid":false,"given":"Justyna","family":"Fercho","sequence":"additional","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"},{"name":"Neurosurgery Department, 10th Military Research Hospital and PolyClinic SPZOZ in Bydgoszcz, 85-681 Bydgoszcz, Poland"},{"name":"Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"},{"name":"Lung Transplant Unit, Cardiac Surgery Department, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0000-7665-5749","authenticated-orcid":false,"given":"Oskar G.","family":"Chasles","sequence":"additional","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bogdan","family":"Jab\u0142o\u0144ski","sequence":"additional","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9165-0679","authenticated-orcid":false,"given":"Jakub","family":"Sobo\u0144","sequence":"additional","affiliation":[{"name":"Neurosurgery Department, 10th Military Research Hospital and PolyClinic SPZOZ in Bydgoszcz, 85-681 Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Przemys\u0142aw","family":"Nag\u00f3rka","sequence":"additional","affiliation":[{"name":"Neurosurgery Department, 10th Military Research Hospital and PolyClinic SPZOZ in Bydgoszcz, 85-681 Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maciej","family":"Mielczarek","sequence":"additional","affiliation":[{"name":"Neurosurgery Department, Stanis\u0142aw Staszic Specialist Hospital, 64-920 Pi\u0142a, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacek","family":"Szypenbejl","sequence":"additional","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"},{"name":"Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8219-2912","authenticated-orcid":false,"given":"Mariusz","family":"Siemi\u0144ski","sequence":"additional","affiliation":[{"name":"Scientific Circle of Neurotraumatology, Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"},{"name":"Department of Emergency Medicine, Medical University of Gda\u0144sk, 80-210 Gda\u0144sk, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jacek","family":"Furtak","sequence":"additional","affiliation":[{"name":"Neurosurgery Department, 10th Military Research Hospital and PolyClinic SPZOZ in Bydgoszcz, 85-681 Bydgoszcz, Poland"},{"name":"Faculty of Medicine, Bydgoszcz University of Science and Technology, 85-796 Bydgoszcz, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2026,5,13]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1007\/s00401-016-1545-1","article-title":"The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A summary","volume":"131","author":"Louis","year":"2016","journal-title":"Acta Neuropathol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"545","DOI":"10.1227\/00006123-198811000-00002","article-title":"Dysembryoplastic neuroepithelial tumor: A surgically curable tumor of young patients with intractable partial seizures: Report of thirty-nine cases","volume":"23","author":"Scheithauer","year":"1988","journal-title":"Neurosurgery"},{"key":"ref_3","first-page":"829","article-title":"The usefulness of MR imaging in the diagnosis of dysembryoplastic neuroepithelial tumor in children: A study of 14 cases","volume":"24","author":"Fernandez","year":"2003","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1068","DOI":"10.1016\/j.clinph.2012.11.015","article-title":"Dysembryoplastic neuroepithelial tumors: Epileptogenicity related to histologic subtypes","volume":"124","author":"Chassoux","year":"2013","journal-title":"Clin. Neurophysiol."},{"key":"ref_5","first-page":"230","article-title":"Dysembryoplastic neuroepithelial tumors: CT, MR findings and imaging follow-up: A study of 53 cases","volume":"28","author":"Cosson","year":"2001","journal-title":"J. Neuroradiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1007\/s003810100496","article-title":"Epidemiology of central nervous system tumors in childhood and adolescence based on the new WHO classification","volume":"17","author":"Rickert","year":"2001","journal-title":"Child\u2019s Nerv. Syst."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Gaviria Carrillo, M., L\u00f3pez, J., Rodr\u00edguez, Q.J.H., Gaona, I., Ortiz-Guerrero, G., and Nava-Mesa, M.O. (2020). Apparent False Lateralization of Seizure Onset by Scalp EEG in Temporal Lobe Epilepsy Associated with Cerebral Cavernous Malformation: A Case Report and Overview. Brain Sci., 10.","DOI":"10.3390\/brainsci10090584"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"492","DOI":"10.3348\/kjr.2012.13.4.492","article-title":"Diffuse Ependymal Dysembryoplastic Neuroepithelial Tumor Causing Spinal Drop Metastases: A Case Report","volume":"13","author":"Kim","year":"2012","journal-title":"Korean J. Radiol."},{"key":"ref_9","first-page":"69","article-title":"Intraventricular dysembryoplastic neuroepithelial tumor-like neoplasm with disseminated spinal tumor","volume":"19","author":"Bilginer","year":"2009","journal-title":"Turk. Neurosurg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1016\/j.radcr.2018.02.014","article-title":"Lethal disseminated dysembryoplastic neuroepithelial tumor following West Nile virus: Report of a very unusual combination","volume":"13","author":"Farkas","year":"2018","journal-title":"Radiol. Case Rep."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"92","DOI":"10.17116\/neiro20248804192","article-title":"Disseminated ependymal dysembryoplastic neuroepithelial tumor: A case report and literature review","volume":"88","author":"Kushel","year":"2024","journal-title":"Zhurnal Vopr. Neirokhirurgii Im. NN Burdenko"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"vi169","DOI":"10.1093\/neuonc\/now212.707","article-title":"RARE-45. Progressive leptomeningeal dissemination of a DNT after west Nile virus meningoencephalitis","volume":"18","author":"Anderson","year":"2016","journal-title":"Neuro-Oncology"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"105","DOI":"10.12688\/f1000research.7404.1","article-title":"West Nile Virus Infection in the Central Nervous System","volume":"5","author":"Winkelmann","year":"2016","journal-title":"F1000Research"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1186\/s13256-023-04062-1","article-title":"Clinicopathological features of dysembryoplastic neuroepithelial tumor: A case series","volume":"17","author":"Rahim","year":"2023","journal-title":"J. Med. Case Rep."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1186\/s40478-020-00898-6","article-title":"FGFR- gene family alterations in low-grade neuroepithelial tumors","volume":"8","author":"Bale","year":"2020","journal-title":"Acta Neuropathol. Commun."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1007\/s00401-016-1549-x","article-title":"Germline and somatic FGFR1 abnormalities in dysembryoplastic neuroepithelial tumors","volume":"131","author":"Rivera","year":"2016","journal-title":"Acta Neuropathol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"174","DOI":"10.4103\/sni.sni_482_16","article-title":"Salvage gamma knife radiosurgery in the management of dysembryoplastic neuroepithelial tumors: Long-term outcome in a single-institution case series","volume":"8","author":"Sinclair","year":"2017","journal-title":"Surg. Neurol. Int."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1177\/1636.17912","article-title":"Radiosurgical options in neuro-oncology: A review on current tenets and future opportunities. Part I: Therapeutic strategies","volume":"100","author":"Ganau","year":"2014","journal-title":"Tumori J."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"57","DOI":"10.5301\/tj.5000215","article-title":"Radiosurgical options in neuro-oncology: A review on current tenets and future opportunities. Part II: Adjuvant radiobiological tools","volume":"101","author":"Ganau","year":"2015","journal-title":"Tumori J."}],"container-title":["Medical Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3271\/14\/2\/250\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T13:37:10Z","timestamp":1778679430000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3271\/14\/2\/250"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,13]]},"references-count":19,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2026,6]]}},"alternative-id":["medsci14020250"],"URL":"https:\/\/doi.org\/10.3390\/medsci14020250","relation":{},"ISSN":["2076-3271"],"issn-type":[{"value":"2076-3271","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,13]]}}}