{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T20:40:31Z","timestamp":1772311231099,"version":"3.50.1"},"reference-count":31,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["SPI"],"published-print":{"date-parts":[[2011,1]]},"abstract":"<jats:p>In this paper, the authors present the case of a patient with multifocal intradural extramedullary ependymoma, and they review 18 previously reported cases.<\/jats:p>\n<jats:p>A 32-year-old man presented to the authors' institution with a 1-month history of partial medullary syndrome. Magnetic resonance imaging of the neuraxis revealed multifocal intradural extramedullary lesions at the bulbomedullary junction and C2\u20133, T5\u201311, L-2, L-4, L-5, and sacrum. Histological examination revealed a WHO Grade II ependymoma.<\/jats:p>\n<jats:p>The literature survey yielded 18 cases of ependymoma at the same location; none of them were multifocal at presentation. The authors analyzed the epidemiological, clinical, and surgical features of all 19 cases reported to date, including the present case.<\/jats:p>\n<jats:p>Patients' ages ranged from 24 to 69 years; 15 patients were women and 4 were men. The time elapsed from symptom onset to diagnosis ranged from 1 month to 8 years. Pain (in 13 patients) and medullary syndrome (in 12) were reported as the initial symptoms (information was not provided for 1 patient). Tumors were predominantly located in the thoracic spine (11), but they also occurred in the cervicothoracic (3), cervical (2), and lumbar (2) spine. The remaining tumor was multifocal. Solitary extramedullary tumors were found intraoperatively in 13 patients; 3 were described as exophytic and 3 as extramedullary with some degree of medullary invasion. Histological examination revealed 9 WHO Grade II tumors, 4 Grade III tumors, and 1 myxopapillary tumor. Data obtained for the remaining cases proved inconclusive. The clinical condition improved in 11 patients, remained stable in 2, and worsened (recurrence or progression) in 6. Of the 4 patients with Grade II tumors who presented with recurrence or neuraxis spreading, 3 had meningeal infiltration or adhesion to the pia mater, which does not rule out the possibility of neoplastic remnants in that area.<\/jats:p>\n<jats:p>Intradural extramedullary ependymomas are rare, they predominate in women in the 5th decade of life, and pain is the most frequent initial symptom. The extent of resection and the presence of meningeal infiltration seem to be key determinants of prognosis. The present case is the first intradural extramedullary ependymoma (with the exception of those occurring at the conus medullaris and terminal filum) with multiple lesions at presentation.<\/jats:p>","DOI":"10.3171\/2010.9.spine09963","type":"journal-article","created":{"date-parts":[[2010,12,8]],"date-time":"2010-12-08T20:26:54Z","timestamp":1291840014000},"page":"65-70","source":"Crossref","is-referenced-by-count":30,"title":["Multifocal intradural extramedullary ependymoma"],"prefix":"10.3171","volume":"14","author":[{"given":"Eduardo Augusto","family":"Iunes","sequence":"first","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jo\u00e3o Norberto","family":"St\u00e1vale","sequence":"additional","affiliation":[{"name":"Pathology, and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"de C\u00e1ssia Caldas Pessoa","sequence":"additional","affiliation":[{"name":"Radiology, Universidade Federal de S\u00e3o Paulo, Brazil"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ricardo","family":"Ansai","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franz Jooji","family":"Onishi","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Manoel Antonio","family":"de Paiva Neto","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ant\u00f4nio","family":"de P\u00e1dua Bonatelli","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S\u00e9rgio","family":"Cavalheiro","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suzana M.","family":"Fleury Malheiros","sequence":"additional","affiliation":[{"name":"Departments of Neurology and Neurosurgery,"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-spine09963","series-title":"Presse Med","first-page":"464","article-title":"Le gliome des fosses nasales. \u00c9tude clinique et anatomo-pathologique","volume":"28","author":"Anglade","year":"1920"},{"key":"b2-spine09963","series-title":"Arch Path","first-page":"584","article-title":"Relation of glioma of the leptomeninges to neuroglia nests: report of a case of astrocytoma of the leptomeninges","volume":"21","author":"Bailey","year":"1936"},{"key":"b3-spine09963","series-title":"Surg Neurol","doi-asserted-by":"publisher","first-page":"536","DOI":"10.1016\/j.surneu.2007.05.055","article-title":"Intradural extramedullary ependymoma: is there constantly a hormonal relationship?","volume":"70","author":"Benzagmout","year":"2008"},{"key":"b4-spine09963","series-title":"Ann Otol Rhinol Laryngol","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1177\/000348942903800209","article-title":"Encephaloma or the so-called nasal glioma","volume":"38","author":"Browder","year":"1929"},{"key":"b5-spine09963","series-title":"J Neurosurg Spine","doi-asserted-by":"publisher","first-page":"476","DOI":"10.3171\/spi.2006.5.5.476","article-title":"Intradural extramedullary spinal anaplastic ependymoma. Case illustration","volume":"5","author":"Cerase","year":"2006"},{"key":"b6-spine09963","series-title":"Ann Acad Med Singapore","first-page":"869","article-title":"Extramedullary thoracic myxopapillary ependymoma\u2014a case report of a rare tumour","volume":"25","author":"Cheng","year":"1996"},{"key":"b7-spine09963","series-title":"Surg Gynecol Obstet","first-page":"183","article-title":"Tumors of the spinal cord; primary extramedullary gliomas","volume":"92","author":"Cooper","year":"1951"},{"key":"b8-spine09963","series-title":"J S Carolina Med Assoc","first-page":"159","article-title":"Congenital ganglio-neuroma of nose, report of a case","volume":"36","author":"Crook","year":"1940"},{"key":"b9-spine09963","series-title":"Spine","doi-asserted-by":"publisher","first-page":"1993","DOI":"10.1097\/00007632-200008010-00021","article-title":"Primary intradural extramedullary ependymoma: case report and review of the literature","volume":"25","author":"Duffau","year":"2000"},{"key":"b10-spine09963","series-title":"Rev Cuba Med Mil","article-title":"Ependimoma intradural extramedular primario","volume":"33","author":"Fuentes Rodr\u00edguez","year":"2004"},{"key":"b11-spine09963","series-title":"Arch Neurobiol (Madr)","first-page":"325","article-title":"[Giant dorsal extramedullary ependymoma.]","volume":"34","author":"Gonz\u00e1lez Feria","year":"1971"},{"key":"b12-spine09963","series-title":"AJNR Am J Neuroradiol","first-page":"818","article-title":"Cystic extramedullary ependymoma","volume":"27","author":"Gra\u00e7a","year":"2006"},{"key":"b13-spine09963","series-title":"Acta Neurochir (Wien)","first-page":"1047","article-title":"Exophytic ependymomas of the spinal cord","volume":"146","author":"Hentschel","year":"2004"},{"key":"b14-spine09963","series-title":"Spine","doi-asserted-by":"publisher","first-page":"2036","DOI":"10.1097\/00007632-199509150-00017","article-title":"Intradural extramedullary ependymoma. A case report","volume":"20","author":"Katoh","year":"1995"},{"key":"b15-spine09963","series-title":"Assoc Res Nerv Ment Dis","first-page":"182","article-title":"Ependymomas: a study of 109 cases","volume":"16","author":"Kernohan","year":"1937"},{"key":"b16-spine09963","series-title":"Acta Radiol","doi-asserted-by":"publisher","first-page":"505","DOI":"10.1177\/028418519103200613","article-title":"MR imaging of spinal intramedullary tumors","volume":"32","author":"Li","year":"1991"},{"key":"b17-spine09963","series-title":"Acta Radiol","doi-asserted-by":"publisher","first-page":"207","DOI":"10.1177\/028418519203300303","article-title":"MR imaging of intradural extramedullary tumors","volume":"33","author":"Li","year":"1992"},{"key":"b18-spine09963","series-title":"Neurosurg Clin N Am","doi-asserted-by":"publisher","first-page":"591","DOI":"10.1016\/S1042-3680(18)30792-7","article-title":"Intradural extramedullary tumors in adults","volume":"1","author":"McCormick","year":"1990"},{"key":"b19-spine09963","series-title":"Neurosurg Clin N Am","doi-asserted-by":"publisher","first-page":"609","DOI":"10.1016\/S1042-3680(18)30793-9","article-title":"Intramedullary tumors in adults","volume":"1","author":"McCormick","year":"1990"},{"key":"b20-spine09963","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"523","DOI":"10.3171\/jns.1990.72.4.0523","article-title":"Intramedullary ependymoma of the spinal cord","volume":"72","author":"McCormick","year":"1990"},{"key":"b21-spine09963","series-title":"Arch Neurobiol (Madr)","first-page":"215","article-title":"[Dorsal extramedullary ependymoma (author's transl).]","volume":"44","author":"Oliver","year":"1981"},{"key":"b22-spine09963","series-title":"J Neurooncol","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1023\/B:NEON.0000041889.71136.62","article-title":"Spinal cord and intradural-extraparenchymal spinal tumors: current best care practices and strategies","volume":"69","author":"Parsa","year":"2004"},{"key":"b23-spine09963","series-title":"J Clin Neurosci","doi-asserted-by":"publisher","first-page":"344","DOI":"10.1016\/S0967-5868(99)90061-9","article-title":"Solitary thoracic intradural extramedullary ependymoma","volume":"6","author":"Payer","year":"1999"},{"key":"b24-spine09963","series-title":"Spine","doi-asserted-by":"publisher","first-page":"E251","DOI":"10.1097\/01.brs.0000161008.13441.7b","article-title":"Intradural extramedullary spinal ependymoma: a benign pathology?","volume":"30","author":"Robles","year":"2005"},{"key":"b25-spine09963","series-title":"Rev Chir","first-page":"147","article-title":"Les fibrogliomes de la r\u00e9gion nasale","volume":"62","author":"Rocher","year":"1924"},{"key":"b26-spine09963","series-title":"Ann d'Anat Path","first-page":"63","article-title":"Les tumeurs m\u00e9ning\u00e9es","volume":"2","author":"Roussy","year":"1925"},{"key":"b27-spine09963","series-title":"J Neurooncol","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1007\/s11060-005-9114-9","article-title":"Spinal extramedullary anaplastic ependymoma with spinal and intracranial metastases","volume":"79","author":"Schuurmans","year":"2006"},{"key":"b28-spine09963","series-title":"J Neuroimaging","doi-asserted-by":"publisher","first-page":"346","DOI":"10.1111\/j.1552-6569.2003.tb00202.x","article-title":"MRI features of intramedullary spinal cord ependymomas","volume":"13","author":"Sun","year":"2003"},{"key":"b29-spine09963","series-title":"J Comput Assist Tomogr","doi-asserted-by":"publisher","first-page":"705","DOI":"10.1097\/00004728-198807000-00045","article-title":"Intradural extramedullary ependymoma: MR-pathologic correlation","volume":"12","author":"Wagle","year":"1988"},{"key":"b30-spine09963","series-title":"Neurosurg Rev","doi-asserted-by":"publisher","first-page":"62","DOI":"10.1007\/BF01111488","article-title":"Intracranial metastasis of a spinal myxopapillary ependymoma. A case report","volume":"21","author":"Woesler","year":"1998"},{"key":"b31-spine09963","series-title":"J Neurosurg","doi-asserted-by":"publisher","first-page":"643","DOI":"10.3171\/jns.1997.87.4.0643","article-title":"Primary extramedullary ependymoma of the thoracic spine. Case illustration","volume":"87","author":"Wolfla","year":"1997"}],"container-title":["Journal of Neurosurgery: Spine"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-spine\/14\/1\/article-p65.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-spine\/14\/1\/article-p65.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-spine\/14\/1\/article-p65.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T22:12:35Z","timestamp":1681596755000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-spine\/14\/1\/article-p65.xml"}},"subtitle":["Case report"],"short-title":[],"issued":{"date-parts":[[2011,1]]},"references-count":31,"journal-issue":{"issue":"1"},"URL":"https:\/\/doi.org\/10.3171\/2010.9.spine09963","relation":{},"ISSN":["1547-5654"],"issn-type":[{"value":"1547-5654","type":"print"}],"subject":[],"published":{"date-parts":[[2011,1]]}}}