{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T06:24:30Z","timestamp":1787293470393,"version":"build-2736575974"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T00:00:00Z","timestamp":1787270400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Intradural extramedullary(IDEM)-type Ependymoma is a rare clinicalentity belonging to the broader category of uncommon glial neoplasms. To date, most published studies have focused predominantly on classic anatomical locations\u2014particularly the filum terminale, whereas the present case arose from an atypical spinal site, highlighting the pronounced anatomical heterogeneity characteristic of this tumor subtype. Moreover, its neuroimaging features demonstrate considerable overlap with those of more common intraspinal tumors, including schwannomas and meningiomas, thereby presenting a substantial diagnostic challenge and elevating the risk of misdiagnosis.increasing the risk of misdiagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Case presentation<\/jats:title>\n                    <jats:p>30-year-old male patient underwent contrast-enhanced lumbar MRI, which revealed a hypervascular intradural tumor spanning the L1-L3 vertebral levels. Complete surgical resection was achieved; the resected specimen measured 4.5 cm\u00d72.5 cm\u00d71.0 cm. Postoperative histopathological examination confirmed the diagnosis of spinal ependymoma. Spinal ependymomas most commonly arise in the filum terminale region, where they are typically amenable to gross-total resection with a relatively low risk of permanent neurological deficit. In this case, however, the tumor was located at the L1-L3 level,a region densely populated by motor neurons and autonomic nerve fibers innervating the lower limbs and pelvic viscera. Consequently, the tumor posed a heightened risk of infiltration into adjacent nerve roots or spinal cord parenchyma. Differential diagnosis:(1) Schwannoma. On MRI, schwannomas are typically iso- to hypointense on T1-weighted images and hyperintense on T2-weighted images. Cystic degeneration is common, and contrast-enhanced imaging generally demonstrates enhancement of the solid component, particularly in Antoni A\u2013predominant areas, whereas cystic regions remain non-enhancing. Intradural extramedullary (IDEM) ependymomas may likewise appear iso- to hypointense on T1-weighted images and iso- to hyperintense on T2-weighted images; however, cystic change, hemorrhage, and heterogeneous enhancement of the solid component may be more prominent. Both lesions can produce neural compression and pain, although radicular pain is more characteristic of schwannoma because of its frequent origin from a spinal nerve root.(2) Spinal meningioma. Spinal meningiomas occur predominantly in middle-aged women and usually appear as well-circumscribed round, oval, or plaque-like intradural extramedullary masses. They are commonly isointense on both T1- and T2-weighted images and demonstrate strong, usually homogeneous enhancement, often accompanied by a dural tail sign. Clinical manifestations may include localized back pain, radicular pain, gait disturbance, and sensory impairment. In contrast, IDEM ependymomas arising in the conus medullaris\u2013cauda equina region may present with more diffuse pain, bilateral lower-extremity weakness, sensory disturbances, or sphincter dysfunction, including urinary retention.(3) Lipoma.Spinal lipomas typically demonstrate homogeneous high signal intensity on T1-weighted images, with complete signal suppression on fat-saturated sequences and little or no contrast enhancement. They are more frequently associated with congenital spinal abnormalities and are commonly detected in children or young patients.(4) Metastatic disease. Spinal ependymomas are generally solitary intradural lesions, often involving the conus medullaris, filum terminale, or cauda equina region, and usually show enhancement of the solid tumor component. By contrast, spinal metastases occur more commonly in older patients and are often associated with a known primary malignancy. Imaging may demonstrate multifocal or noncontiguous vertebral marrow replacement, low signal intensity on T1-weighted images, contrast enhancement, cortical destruction, and possible epidural or paraspinal extension. Nevertheless, intradural metastasis should also be considered when a patient has a history of systemic cancer or evidence of disseminated disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This case report describes a rare multi-segmental lumbar intradural extramedullary ependymoma located at the atypical L1\u2013L3 spinal levels, emphasizing the diagnostic and therapeutic challenges associated with non-classical anatomical presentations. Unlike the more commonly reported filum terminale ependymomas, tumors arising in the upper lumbar region are closely related to cauda equina nerve roots, motor pathways, and autonomic neural structures, thereby requiring a surgical strategy that balances maximal tumor resection with preservation of neurological function. In line with current international perspectives, contrast-enhanced MRI should be regarded as the first-line modality for preoperative localization, assessment of tumor enhancement patterns, and evaluation of its relationship with adjacent neural structures; however, definitive diagnosis still depends on integrated histopathological, immunohistochemical, and molecular evaluation. In the present case, gross-total resection was achieved with preservation of neurological function, and no evidence of recurrence was observed during follow-up, supporting the view that surgery remains the cornerstone of treatment for low-grade spinal ependymoma, while adjuvant radiotherapy should be considered mainly in cases of subtotal resection, residual disease, recurrence, or higher-grade pathology. More importantly, this case highlights the need to overcome location-based diagnostic bias, include ependymoma in the differential diagnosis of atypical lumbar intradural extramedullary tumors, and adopt a multidisciplinary, function-preserving, precision-based management strategy. Long-term radiological surveillance remains essential because recurrence may occur even after apparently complete resection. Therefore, this report provides clinically relevant evidence for improving recognition, individualized surgical planning, and postoperative follow-up strategies for rare lumbar IDEM ependymomas.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fonc.2026.1906737","type":"journal-article","created":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T05:38:47Z","timestamp":1787290727000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["A rare case of multi-segmental lumbar intradural ependymoma: clinical presentation and surgical management: a case report"],"prefix":"10.3389","volume":"16","author":[{"given":"Tao","family":"Chen","sequence":"first","affiliation":[{"name":"The First Clinical Medical College of China Three Gorges University","place":["Yichang, Hubei, China"]},{"name":"Spinal Surgery Department, Yichang Central People\u2019s Hospital","place":["Yichang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianfeng","family":"Chen","sequence":"additional","affiliation":[{"name":"The First Clinical Medical College of China Three Gorges University","place":["Yichang, Hubei, China"]},{"name":"Spinal Surgery Department, Yichang Central People\u2019s Hospital","place":["Yichang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bin","family":"Wu","sequence":"additional","affiliation":[{"name":"The First Clinical Medical College of China Three Gorges University","place":["Yichang, Hubei, China"]},{"name":"Spinal Surgery Department, Yichang Central People\u2019s Hospital","place":["Yichang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weijin","family":"Li","sequence":"additional","affiliation":[{"name":"The First Clinical Medical College of China Three Gorges University","place":["Yichang, Hubei, China"]},{"name":"Spinal Surgery Department, Yichang Central People\u2019s Hospital","place":["Yichang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fan","family":"Zhang","sequence":"additional","affiliation":[{"name":"The First Clinical Medical College of China Three Gorges University","place":["Yichang, Hubei, China"]},{"name":"Spinal Surgery Department, Yichang Central People\u2019s Hospital","place":["Yichang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jie","family":"Lin","sequence":"additional","affiliation":[{"name":"The Second People\u2019s Hospital of China Three Gorges University","place":["Yichang, Hubei, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,8,21]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.3389\/fonc.2023.1301179","article-title":"Spinal ependymoma in adults: from molecular advances to new treatment perspectives","volume":"13","author":"Cerretti","year":"2023","journal-title":"Front Oncol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nox166","article-title":"EANO guidelines for the diagnosis and treatment of ependymal tumors","volume":"20","author":"Rud\u00e0","year":"2018","journal-title":"Neuro Oncol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.2176\/nmccrj.cr.2020-0354","article-title":"A case of spinal ependymoma developed in the extramedullary location: a case report and literature review","volume":"8","author":"Makino","year":"2021","journal-title":"NMC Case Rep J"},{"key":"B4","doi-asserted-by":"publisher","first-page":"995","DOI":"10.1080\/17434440.2021.1977123","article-title":"Comparison of clinical effectiveness of fenestrated and conventional pedicle screws in patients undergoing spinal surgery: a systematic review and meta-analysis","volume":"18","author":"Yagi","year":"2021","journal-title":"Expert Rev Med Devices"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.14245\/ns.2346388.194","article-title":"Spinal cord subependymoma: a subanalysis of the neurospinal society of Japan multicenter study of intradural spinal cord tumors","volume":"20","author":"Yagi","year":"2023","journal-title":"Neurospine"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.7759\/cureus.49086","article-title":"Spinal ependymomas: an updated WHO classification and a narrative review","volume":"15","author":"Villanueva-Castro","year":"2023","journal-title":"Cureus"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1007\/s11912-022-01260-w","article-title":"Ependymoma: evaluation and management updates","volume":"24","author":"Rud\u00e0","year":"2022","journal-title":"Curr Oncol Rep"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1038\/s41568-021-00433-2","article-title":"The biology of ependymomas and emerging novel therapies","volume":"22","author":"Saleh","year":"2022","journal-title":"Nat Rev Cancer"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1038\/nature25169","article-title":"Therapeutic targeting of ependymoma as informed by oncogenic enhancer profiling","volume":"553","author":"Mack","year":"2018","journal-title":"Nature"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.3390\/jcm14051671","article-title":"Systematic review comparing open versus minimally invasive surgical management of intradural extramedullary tumours (IDEM)","volume":"14","author":"Baig Mirza","year":"2025","journal-title":"J Clin Med"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1111\/bpa.13068","article-title":"Updates in the classification of ependymal neoplasms: the 2021 WHO classification and beyond","volume":"32","author":"Kresbach","year":"2022","journal-title":"Brain Pathol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"S4","DOI":"10.1093\/neuonc\/noab183","article-title":"Utilizing preclinical models to develop targeted therapies for rare central nervous system cancers","volume":"23","author":"Arakaki","year":"2021","journal-title":"Neuro Oncol"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noab183","article-title":"Ependymoma and chordoma","volume":"87","author":"Levine","year":"2020","journal-title":"Neurosurgery"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1016\/j.cancergen.2025.02.011","article-title":"MicroRNA dysregulation and target genes in common spinal tumors","author":"Oliaee","year":"2025","journal-title":"Cancer Genet"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.3390\/cancers13246218","article-title":"Molecular classification and therapeutic targets in ependymoma","volume":"13","author":"Larrew","year":"2021","journal-title":"Cancers (Basel)"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcancer.2022.06.011","article-title":"Benefits and limitations of nanomedicine treatment of brain cancers and age-dependent neurodegenerative disorders","volume":"86","author":"Neganova","year":"2022","journal-title":"Semin Cancer Biol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1093\/neuonc\/noae186","article-title":"Central nervous system tumors in adolescents and young adults: a Society for Neuro-Oncology Consensus Review on diagnosis, management, and future directions","volume":"27","author":"Lim-Fat","year":"2025","journal-title":"Neuro Oncol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1016\/j.jclinepi.2013.05.015","article-title":"The CARE guidelines: consensus-based clinical case reporting guideline development","volume":"66","author":"Gagnier","year":"2013","journal-title":"J Clin Epidemiol"},{"key":"B19","doi-asserted-by":"publisher","first-page":"105747","DOI":"10.1016\/j.ijscr.2021.105747","article-title":"Surgical management of a huge intramedullary ependymoma from oblongata to T4: A case report","volume":"81","author":"Zhang","year":"2021","journal-title":"Int J Surg Case Rep"},{"key":"B20","doi-asserted-by":"publisher","first-page":"e122833","DOI":"10.5812\/ijcm-122833","article-title":"Massive intramedullary ependymoma: A case report","volume":"16","author":"Mirbolook","year":"2023","journal-title":"Int J Cancer Man"},{"key":"B21","doi-asserted-by":"publisher","first-page":"e20329","DOI":"10.7759\/cureus.20329","article-title":"Multisegment intradural extramedullary ependymoma","volume":"13","author":"Trivedi","year":"2021","journal-title":"Cureus"},{"key":"B22","doi-asserted-by":"publisher","first-page":"e20277","DOI":"10.7759\/cureus.20277","article-title":"Chronic pain secondary to a cervical intramedullary ependymoma: A case report","volume":"13","author":"Atoot","year":"2021","journal-title":"Cureus"},{"key":"B23","doi-asserted-by":"publisher","first-page":"170","DOI":"10.1016\/j.jcm.2022.01.004","article-title":"Bilateral lumbar radiculopathy secondary to myxopapillary ependymoma: A case report","volume":"20","author":"D'Angiolillo","year":"2021","journal-title":"J Chiropratic Med"},{"key":"B24","doi-asserted-by":"publisher","first-page":"CASE22141","DOI":"10.3171\/CASE22141","article-title":"Multifocal intradural extramedullary ependymoma, MYCN amplified: Illustrative case","volume":"3","author":"Shields","year":"2022","journal-title":"J Neosurg: Case Lessons"},{"key":"B25","doi-asserted-by":"publisher","first-page":"150","DOI":"10.5455\/medarh.2023.77.150-154","article-title":"Myxopapillary ependymoma\u2014a case report of rare multicentric subtype and literature review","volume":"77","author":"Almatrafi","year":"2023","journal-title":"Med Arch"},{"key":"B26","doi-asserted-by":"publisher","first-page":"e39381","DOI":"10.7759\/cureus.39381","article-title":"Spinal myxopapillary ependymoma: A rare case and review of management strategies","volume":"15","author":"Soto","year":"2023","journal-title":"Cureus"},{"key":"B27","doi-asserted-by":"publisher","first-page":"1414","DOI":"10.3348\/jksr.2022.0173","article-title":"Intradural extramedullary ependymoma with hemorrhage: A case report","volume":"84","author":"Choi","year":"2023","journal-title":"J Korean Soc Rad"},{"key":"B28","doi-asserted-by":"publisher","first-page":"e942392","DOI":"10.12659\/AJCR.942392","article-title":"Giant myxopapillary ependymoma with multi-site neural axis metastases: A rare case with suboptimal outcome","volume":"25","author":"Balodis","year":"2024","journal-title":"Am J Case Rep"},{"key":"B29","doi-asserted-by":"publisher","first-page":"1489","DOI":"10.1016\/j.radcr.2023.12.010","article-title":"Unusual location of myxopapillary ependymoma in the sacrum: Case report and review of the literature","volume":"19","author":"Kouhen","year":"2024","journal-title":"Rad Case Rep"},{"key":"B30","doi-asserted-by":"publisher","first-page":"e87616","DOI":"10.7759\/cureus.87616","article-title":"Spinal myxopapillary ependymoma presenting with isolated intracranial hypertension: A case report","volume":"17","author":"Oke","year":"2025","journal-title":"Cureus"},{"key":"B31","doi-asserted-by":"publisher","first-page":"e84996","DOI":"10.7759\/cureus.84996","article-title":"Myxopapillary ependymoma: A case report","volume":"17","author":"Arjomand","year":"2025","journal-title":"Cureus"},{"key":"B32","doi-asserted-by":"publisher","first-page":"e41210","DOI":"10.1097\/MD.0000000000041210","article-title":"Intradural extramedullary double primary ependymoma and meningioma: Rare condition, case report and literature review","volume":"104","author":"Yuan","year":"2025","journal-title":"Medicine"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1906737\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T05:38:48Z","timestamp":1787290728000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2026.1906737\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,21]]},"references-count":32,"alternative-id":["10.3389\/fonc.2026.1906737"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2026.1906737","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,21]]},"article-number":"1906737"}}