{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,2]],"date-time":"2026-07-02T02:41:59Z","timestamp":1782960119689,"version":"3.54.5"},"reference-count":40,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"14","license":[{"start":{"date-parts":[[2023,4,3]],"date-time":"2023-04-03T00:00:00Z","timestamp":1680480000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,4,3]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Skull base chordomas are typically extradural and present with cranial nerve deficits, headache, and visual disturbances. Clival chordoma involving the dura and presenting as a spontaneous cerebrospinal fluid (CSF) leak is extremely rare and can be mistaken for other skull base lesions. Here the authors present a case of chordoma with an unusual presentation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>A 43-year-old female who presented with clear nasal drainage was diagnosed with CSF rhinorrhea secondary to a clival defect previously thought to be ecchordosis physaliphora. The patient subsequently developed bacterial meningitis and underwent endoscopic, endonasal, transclival gross-total resection of the lesion with repair of the dural defect. Pathology revealed brachyury-positive chordoma. She received adjuvant proton beam radiotherapy and has remained stable for 2 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>Spontaneous CSF rhinorrhea can occur as a rare primary presentation of clival chordoma, requiring careful radiological interpretation and a high index of suspicion for diagnosis. Chordoma cannot be reliably differentiated from benign notochordal lesions based on imaging alone; thus, intraoperative exploration and immunohistochemistry play key roles. Clival lesions presenting with CSF rhinorrhea should undergo prompt resection to facilitate diagnosis and prevent complications. Future studies on connections between chordoma and benign notochordal lesions may help to establish management guidelines.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case2347","type":"journal-article","created":{"date-parts":[[2023,4,3]],"date-time":"2023-04-03T17:28:17Z","timestamp":1680542897000},"source":"Crossref","is-referenced-by-count":3,"title":["Chronic cerebrospinal fluid rhinorrhea as an initial presentation of chordoma: illustrative case"],"prefix":"10.3171","volume":"5","author":[{"given":"Kiana Y.","family":"Prather","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen H.","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kibwei A.","family":"McKinney","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ian F.","family":"Dunn","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma; and"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1754","reference":[{"key":"b1","series-title":"Chordoma","author":"Tenny S","year":"2022","unstructured":"Tenny S, Varacallo M. Chordoma. StatPearls; 2022."},{"issue":"3","key":"b2","series-title":"Neurosurg Clin N Am","first-page":"437","article-title":"Chordomas: a review","volume":"26","author":"George B","year":"2015","unstructured":"George B, Bresson D, Herman P, Froelich S. Chordomas: a review. Neurosurg Clin N Am. 2015;26(3):437\u2013452.26141362"},{"issue":"3","key":"b3","series-title":"Neurosurg Focus","first-page":"E1","article-title":"Chordomas of the skull base: follow-up review and prognostic factors","volume":"10","author":"Colli BO","year":"2001","unstructured":"Colli BO, Al-Mefty O. Chordomas of the skull base: follow-up review and prognostic factors. Neurosurg Focus. 2001;10(3):E1.16734401"},{"key":"b4","series-title":"Cerebrospinal Fluid Leak","author":"Severson M","year":"2022","unstructured":"Severson M, Strecker-McGraw MK. Cerebrospinal Fluid Leak. StatPearls; 2022."},{"key":"b5","series-title":"J Med Case Reports","first-page":"410","article-title":"Clival chordoma with an atypical presentation: a case report","volume":"6","author":"Alshammari J","year":"2012","unstructured":"Alshammari J, Monnier P, Daniel RT, Sandu K. Clival chordoma with an atypical presentation: a case report. J Med Case Reports. 2012;6:410."},{"issue":"5","key":"b6","series-title":"Br J Neurosurg","first-page":"549","article-title":"An unusual presentation of clival chordoma: a case report and review of the literature","volume":"34","author":"Andijani M","year":"2020","unstructured":"Andijani M, Jamjoom A, Togersen A, Ram B, Bodkin P, Kamel M. An unusual presentation of clival chordoma: a case report and review of the literature. Br J Neurosurg. 2020;34(5):549\u2013551.31226887"},{"issue":"8","key":"b7","series-title":"J Clin Neurosci","first-page":"1083","article-title":"Transclival cerebrospinal fluid rhinorrhea as the initial presenting symptom of a tiny intradural chordoma","volume":"17","author":"Feng K","year":"2010","unstructured":"Feng K, Qiuhang Z, Qiuyi Q. Transclival cerebrospinal fluid rhinorrhea as the initial presenting symptom of a tiny intradural chordoma. J Clin Neurosci. 2010;17(8):1083\u20131085.20554205"},{"issue":"4","key":"b8","series-title":"Neurol India","first-page":"513","article-title":"Spontaneous cerebrospinal fluid rhinorrhea: a rare initial presentation of clival chordoma","volume":"57","author":"Hsieh CT","year":"2009","unstructured":"Hsieh CT, Liu MY, Su WF, Ju DT. Spontaneous cerebrospinal fluid rhinorrhea: a rare initial presentation of clival chordoma. Neurol India. 2009;57(4):513\u2013514.19770569"},{"issue":"1","key":"b9","series-title":"Neurocirugia (Astur: Engl Ed)","first-page":"41","article-title":"Spontaneous CSF rhinorrhea as a presenting symptom of a clival chordoma","volume":"32","author":"Quiroz Tejada AR","year":"2021","unstructured":"Quiroz Tejada AR, Simal-Julian JA, Perez de San Roman-Mena L, Miranda-Lloret P, Botella-Asuncion C, Sanchis Martin MR. Spontaneous CSF rhinorrhea as a presenting symptom of a clival chordoma. Neurocirugia (Astur: Engl Ed). 2021;32(1):41\u201343.32001132"},{"issue":"6","key":"b10","series-title":"Skull Base","first-page":"395","article-title":"Cerebrospinal fluid rhinorrhea secondary to ecchordosis physaliphora","volume":"18","author":"Alli A","year":"2008","unstructured":"Alli A, Clark M, Mansell NJ. Cerebrospinal fluid rhinorrhea secondary to ecchordosis physaliphora. Skull Base. 2008;18(6):395\u2013399.19412409"},{"key":"b11","series-title":"Surg Neurol Int","first-page":"13","article-title":"A case of ecchordosis physaliphora presenting with an abducens nerve palsy: a rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery","volume":"4","author":"Yamamoto T","year":"2013","unstructured":"Yamamoto T, Yano S, Hide T, Kuratsu J. A case of ecchordosis physaliphora presenting with an abducens nerve palsy: a rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery. Surg Neurol Int. 2013;4:13.23493306"},{"issue":"4","key":"b12","series-title":"J Neurosurg Pediatr","first-page":"328","article-title":"Giant ecchordosis physaliphora in an adolescent girl: case report","volume":"12","author":"Krisht KM","year":"2013","unstructured":"Krisht KM, Palmer CA, Osborn AG, Couldwell WT. Giant ecchordosis physaliphora in an adolescent girl: case report. J Neurosurg Pediatr. 2013;12(4):328\u2013333."},{"issue":"2","key":"b13","series-title":"Neurosurgery","first-page":"E387","article-title":"Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report","volume":"64","author":"Ciarpaglini R","year":"2009","unstructured":"Ciarpaglini R, Pasquini E, Mazzatenta D, Ambrosini-Spaltro A, Sciarretta V, Frank G. Intradural clival chordoma and ecchordosis physaliphora: a challenging differential diagnosis: case report. Neurosurgery. 2009;64(2):E387\u2013E388, discussion E388.19190444"},{"issue":"3","key":"b14","series-title":"Cureus","first-page":"e547","article-title":"Proposed diagnostic criteria, classification schema, and review of literature of notochord-derived ecchordosis physaliphora","volume":"8","author":"Lagman C","year":"2016","unstructured":"Lagman C, Varshneya K, Sarmiento JM, Turtz AR, Chitale RV. Proposed diagnostic criteria, classification schema, and review of literature of notochord-derived ecchordosis physaliphora. Cureus. 2016;8(3):e547.27158576"},{"issue":"8","key":"b15","series-title":"Arch Pathol Lab Med","first-page":"1170","article-title":"The prognostic value of Ki-67, p53, epidermal growth factor receptor, 1p36, 9p21, 10q23, and 17p13 in skull base chordomas","volume":"134","author":"Horbinski C","year":"2010","unstructured":"Horbinski C, Oakley GJ, Cieply K,  The prognostic value of Ki-67, p53, epidermal growth factor receptor, 1p36, 9p21, 10q23, and 17p13 in skull base chordomas. 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Acta Neurochir (Wien). 2001;143(10):1065\u20131069."},{"issue":"3","key":"b18","series-title":"World Neurosurg","first-page":"194","article-title":"Intradural cranial chordoma","volume":"73","author":"Ito E","year":"2010","unstructured":"Ito E, Saito K, Nagatani T,  Intradural cranial chordoma. World Neurosurg. 2010;73(3):194\u2013197, e31.20860957"},{"issue":"3","key":"b19","series-title":"Clin Neuropathol","first-page":"169","article-title":"Primary intradural chordoma: report on three cases and review of the literature","volume":"29","author":"Bergmann M","year":"2010","unstructured":"Bergmann M, Abdalla Y, Neubauer U, Schildhaus HU, Probst-Cousin S. Primary intradural chordoma: report on three cases and review of the literature. 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