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"Chordoma"

Technical Note

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Endoscopic Endonasal Transnasopharyngeal Approach for Ventral Craniovertebral Junction Lesions: A Technical Note
Neurospine. 2025;22(3):737-747.   Published online September 30, 2025
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Endoscopic Endonasal Transnasopharyngeal Approach for Ventral Craniovertebral Junction Lesions: A Technical Note
Neurospine. 2025;22(3):737-747.   Published online September 30, 2025
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Objective
Lesions of the ventral craniovertebral junction are difficult to access owing to their deep location and proximity to critical neurovascular and pharyngeal structures. In this study, we aimed to describe the surgical technique and clinical outcomes of the endoscopic endonasal transnasopharyngeal approach for ventral craniovertebral junction lesions and highlight key considerations regarding approach selection, airway management, and occipitocervical stabilization.
Methods
We retrospectively reviewed 7 patients who underwent the endoscopic endonasal transnasopharyngeal approach for ventral craniovertebral junction lesions. The analysis included preoperative planning for surgical access, intraoperative technique, postoperative management, airway and nutritional strategies, and the need for occipitocervical fixation. One representative case is presented to illustrate key technical steps.
Results
Of the 7 patients, 6 had neoplastic lesions and 1 had basilar invagination. Despite a relatively large mean lesion size of 39.4 mm, subtotal or greater resection was achieved in 5 of the 6 tumor cases. Occipitocervical fixation was performed in 2 cases. Two patients underwent prophylactic tracheostomy because of anticipated airway compromise. Of the 5 orally intubated cases, 3 were extubated immediately and 2 by postoperative day 2. Oral feeding resumed by day 10 in 6 cases. No postoperative infections or cerebrospinal fluid leakage occurred. One patient experienced transient velopharyngeal insufficiency, which resolved spontaneously.
Conclusion
The endoscopic endonasal transnasopharyngeal approach is a safe and effective option for ventral craniovertebral junction lesions when appropriately selected. Careful preoperative evaluation and individualized management of airway and spinal stability are essential for favorable outcomes.
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  • 1 Web of Science

Original Article

Regular Issue

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The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study
Neurospine. 2024;21(3):942-953.   Published online September 30, 2024
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The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study
Neurospine. 2024;21(3):942-953.   Published online September 30, 2024
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Objective
Chordoma, a rare malignant tumor originating from embryonal notochord remnants, exhibits high resistance to conventional treatments, making surgical resection imperative. However, the factors influencing prognosis specifically for cervical spine chordoma have not been clearly identified. We investigate the prognosis of cervical spine chordoma with factors influential in a nationwide multicenter retrospective study.
Methods
This study included all patients diagnosed with cervical spine chordoma at 7 tertiary referral centers from January 1998 to March 2023, excluding those with clivus and thoracic spine chordomas extending into the cervical spine. Local recurrence (LR) was identified through follow-up magnetic resonance imaging, either as reappearance in completely resected tumors or regrowth in residual tumors. The study assessed LR and overall survival, analyzing factors influencing LR and death.
Results
Forty-five patients with cervical spine chordoma had a mean age of 46.4 years. Over a median follow-up of 52 months, LR and distant metastasis were observed in 21 (46.7%) and 4 patients (8.9%), respectively, and 16 patients (36%) were confirmed dead. The 5-year and 10-year cumulative LR rates were 51.3% and 60%, respectively, while the 5-year and 10-year survival rates were 82% and 53%. Age was the only significant factor affecting mortality (hazard ratio, 1.04; 95% confidence interval, 1.04–1.07; p=0.015). Notably, the degree of resection and adjuvant therapy did not statistically significantly impact local tumor control and mortality.
Conclusion
This study, the largest multicenter retrospective analysis of cervical spine chordoma in Korea, identified age as the only factor significantly affecting patient survival.

Citations

Citations to this article as recorded by  Crossref logo
  • Cervical Vertebral Body Implant Modification Accommodating Vertebral Artery Aneurysm Clips: A Case Report
    Robert Rothrock, Vitaly Siomin, Rupesh Kotecha, Starlie C Belnap, Michael McDermott
    Cureus.2026;[Epub]     CrossRef
  • SURGICAL MANAGEMENT OF ANTERIORLY LOCATED TUMORS AT THE CRANIOVERTEBRAL JUNCTION: ADVANCES AND CHALLENGES
    ANTONIO VINICIUS DA SILVA GONÇALVES DA ROCHA, MARIANA CHANTRE-JUSTINO, OCTAVIO AUGUSTO TOMÉ DA SILVA, DAVI SOÉJIMA CORREIA RAMALHO, ALDERICO GIRÃO CAMPOS DE BARROS, ULLYANOV TOSCANO, LUIS E. CARELLI
    Coluna/Columna.2025;[Epub]     CrossRef
  • Comparative Outcomes of Brachyury Vaccine vs. Imatinib in Advanced Chordoma: A Mayo Clinic Experience
    Juan P. Navarro-Garcia de Llano, Harshvardhan G. Iyer, Harry C. Hoffman, Mahesh Seetharam, Steven Attia, Oluwaseun O. Akinduro
    Cancers.2025; 17(21): 3493.     CrossRef
  • 11,369 View
  • 171 Download
  • 2 Web of Science
  • 3 Crossref

Technical Note

Spine and Spinal Cord Tumors DSPN-Neurospine Special Issue

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Sacral Nerves Reconstruction After Surgical Resection of a Large Sacral Chordoma Restores the Urinary and Sexual Function and the Anal Continence
Neurospine. 2022;19(1):155-162.   Published online January 30, 2022
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Sacral Nerves Reconstruction After Surgical Resection of a Large Sacral Chordoma Restores the Urinary and Sexual Function and the Anal Continence
Neurospine. 2022;19(1):155-162.   Published online January 30, 2022
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Objective
Chordomas are slow-growing tumors, with a high tendency to local relapse. En bloc resection is related to the most favorable outcome in terms of survival but is frequently associated with permanent neurological deficits involving sphincters and sexual functions. In the present article, we describe an innovative technique of en bloc resection followed by reconstruction of the sacral nerves with nerve grafts.
Methods
The chordoma was excised through a posterior approach after dividing the proximal and distal sacral nerves using the established technique. After that, a microsurgical S2-S3-S4 nerve reconstruction was performed connecting the proximal and distal stumps with sural nerve grafts withdrawn from both lower limbs.
Results
Immediately after surgery, the patient experienced complete impairment of sexual function and sphincters with urinary and fecal incontinence. After 6 months, there was a progressive recovery of sexual function and sphincter control. One year after the operation, the patient achieved an adequate sexual life (erection and ejaculation) and complete control of the bladder and anal sphincter.
Conclusion
Reconstruction of nerves sacrificed during sacral tumor removal has been shown to be effective in restoring sphincter and sexual function and is a promising technique that may significantly improve patients' quality of life.

Citations

Citations to this article as recorded by  Crossref logo
  • Characterization of Soft Tissue Reconstruction Following Chordoma Resection
    Shanmuga Priya Rajagopalan, Danielle Sim, Waldemar A. Rodriguez-Silva, Ananya Dewan, Siam Rezwan, Pritika Papali, Abdel-Hameed Al-Mistarehi, Andrew Hersh, Daniel Lubelski, Carisa M. Cooney, Salih Colakoglu
    Journal of Reconstructive Microsurgery.2026; 42(03): 242.     CrossRef
  • Clinical Anatomy of the Sacral Nerve Roots and Its Relevance to Their Reconstruction After Sacrectomy
    Radek Kaiser, Anhelina Khadanovich, Michal Benes, Jeremy Reynolds, Gerard Mawhinney, Henk Giele, David Kachlik
    Neurosurgery.2025; 96(3): 505.     CrossRef
  • Total resection of sacral giant cell tumor with en bloc resection, sacral nerve reconstruction, and custom 3D-printed implant: a case report with long-term follow-up
    Dong Hu, Fei Song, Songhua Xiao
    International Journal of Surgery Case Reports.2025;[Epub]     CrossRef
  • Multidisciplinary En-Bloc Resection of Sacral Chordoma: A Narrative Review and Illustrative Case
    Daniel Kiss-Bodolay, Frederic Ris, Adrien Lavalley, Aria Nouri, Carlo M. Oranges, Guillaume Meurette, Karl Schaller, Enrico Tessitore, Granit Molliqaj
    Journal of Clinical Medicine.2025; 14(13): 4480.     CrossRef
  • Impact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma
    Furkan Erdoğan, Alparslan Yurtbay, Bedirhan Albayrak, Tolgahan Cengiz, Nevzat Dabak
    Journal of Clinical Medicine.2025; 14(15): 5464.     CrossRef
  • Navigation-guided resection of sacrococcygeal tumors: Case series with clinical and functional outcomes
    Hangeul Park, Woojin Kim, Jungbo Sim, Ho Sung Myeong, Seunghoon Lee, Jun-Hoe Kim, Chang-Hyun Lee, Chi Heon Kim
    Medicine.2025; 104(43): e45587.     CrossRef
  • Nerve transfer for restoration of lower motor neuron-lesioned bladder, urethral and anal sphincter function. Part 4: Effectiveness of the motor reinnervation
    Ekta Tiwari, Danielle S. Porreca, Alan S. Braverman, Lewis Holt-Bright, Nagat A. Frara, Justin M. Brown, Benjamin R. Johnston, Stanley F. Bazarek, Brendan A. Hilliard, Michael Mazzei, Michel A. Pontari, Daohai Yu, Michael R. Ruggieri, Mary F. Barbe
    American Journal of Physiology-Regulatory, Integrative and Comparative Physiology.2024; 326(6): R528.     CrossRef
  • Cordoma sacrococcígeo: revisión del tema
    Luis Jair Sánchez Torres, Héctor Alejandro Pulido Torres, Absalón Espinoza Velazco, Perla Lidia Pérez Pineda, María Magdalena Chávez Reyna
    Cirugía de Columna.2024; 2(2): 104.     CrossRef
  • Reply to Commentary on “Sacral Nerves Reconstruction After Surgical Resection of a Large Sacral Chordoma Restores the Urinary and Sexual Function and the Anal Continence”
    Luigi Valentino Berra
    Neurospine.2022; 19(4): 1132.     CrossRef
  • Commentary on “Sacral Nerves Reconstruction After Surgical Resection of a Large Sacral Chordoma Restores the Urinary and Sexual Function and the Anal Continence”
    Zhuolin Dai, Weiyang Zhong
    Neurospine.2022; 19(4): 1130.     CrossRef
  • 9,312 View
  • 367 Download
  • 9 Web of Science
  • 10 Crossref

Original Article

APCSS special Topic-Craniovertebral Junction Surgery

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Suture Repair in Endoscopic Surgery for Craniovertebral Junction
Neurospine. 2019;16(2):257-266.   Published online June 30, 2019
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Suture Repair in Endoscopic Surgery for Craniovertebral Junction
Neurospine. 2019;16(2):257-266.   Published online June 30, 2019
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Objective
Endoscopic approaches to the craniovertebral junction (CVJ) have been established as viable and effective surgical treatments in the past decade. One of the major complications is leakage of the cerebrospinal fluid (CSF). This study aimed to investigate the efficacy and feasibility of suture closure at the nasopharyngeal mucosa upon durotomy.
Methods
A series of consecutive patients who underwent different endoscopic approaches to the CVJ were retrospectively reviewed. The pathologies, surgical corridors, neurological and functional outcomes, radiological evaluations, and complications were analyzed. Different strategies of repair for the intraoperative CSF leakage were described and compared.
Results
A total of 22 patients covering 13 years were analyzed. There were 12, 2, and 8 patients who underwent transnasal, transoral, and combined approaches, respectively. There were 8 patients (36.4%) who experienced intraoperative CSF leakage, and were grouped into 2: 4 in the nonsuture (NS) group and 4 in the suture-repaired (SR) group. The NS group had 3 (75%) persistent CSF leakages postoperation that caused 1 mortality, whereas patients of the SR group had only 1 minor CSF rhinorrhea that healed spontaneously within days.
Conclusion
In this series of 22 patients who required anterior endoscopic resection of pathologies at the CVJ, there was 1 (4.5%) serious complication related to CSF leakage. For patients who had no durotomy, the mucosal incision at the nasopharynx usually healed rapidly and there were few procedure-related complications. For patients with intraoperative CSF leakage, suture closure was technically challenging but could significantly lower the risks of postoperative complications.

Citations

Citations to this article as recorded by  Crossref logo
  • Surgical treatment and clinical outcome in non-inflammatory atlantoaxial degeneration and retro-odontoid pseudotumor
    Raimunde Liang, Bernhard Meyer, Vicki M. Butenschoen
    Brain and Spine.2025; 5: 105621.     CrossRef
  • Navigated Anterior Full-Endoscopic Transcervical Approach Odontoidectomy for Traumatic Posterior Atlantoaxial Dislocation Without Odontoid Fracture
    Juan Felipe Abaunza-Camacho, Sara Gomez-Niebles, Humberto Madrinan-Navia, Alberto Daza-Ovalle, Natalia Guevara-Moriones, Mario Fernando Rodríguez, Jorge Torres Mancera, Camilo Peña, William Mauricio Riveros-Castillo, Javier M. Saavedra
    Operative Neurosurgery.2024; 27(5): 641.     CrossRef
  • Minimally invasive surgery for invaginated CII odontoid process
    A.N. Shkarubo, I.V. Chernov, D.N. Andreev, N.A. Konovalov, M.E. Sinelnikov
    Burdenko's Journal of Neurosurgery.2023; 87(3): 5.     CrossRef
  • Comparative analysis of endoscopic transnasal and microsurgical transoral odontoidectomy: Literature review and own experience
    Alexey N. Shkarubo, Anton G. Nazarenko, Ilya V. Chernov, Dmitry N. Andreev, Alexandr A. Kuleshov, Nikolai A. Konovalov, Igor N. Lisyanskiy, Mikhail E. Sinelnikov
    N.N. Priorov Journal of Traumatology and Orthopedics.2023; 30(1): 41.     CrossRef
  • Endoscopic Transnasal Odontoidectomy for Ventral Decompression of the Craniovertebral Junction: Surgical Technique and Clinical Outcome in a Case Series of 19 Patients
    Vicki M Butenschoen, Maria Wostrack, Bernhard Meyer, Jens Gempt
    Operative Neurosurgery.2021; 20(1): 24.     CrossRef
  • Nonrheumatoid Retro-Odontoid Pseudotumors: Characteristics, Surgical Outcomes, and Time-Dependent Regression After Posterior Fixation
    Ryoko Niwa, Keisuke Takai, Makoto Taniguchi
    Neurospine.2021; 18(1): 177.     CrossRef
  • 10,243 View
  • 148 Download
  • 4 Web of Science
  • 6 Crossref

Case Reports

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Two Cases of Dedifferentiated Chordoma in the Sacrum
Korean J Spine. 2015;12(3):230-234.   Published online September 30, 2015
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Two Cases of Dedifferentiated Chordoma in the Sacrum
Korean J Spine. 2015;12(3):230-234.   Published online September 30, 2015
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Dedifferentiated chordoma (DC) is defined as a chordoma containing sarcoma components. DC is distinguished from conventional chordoma by the rapidity of tumor growth and the potential for distant metastasis. We report two cases of DC, which are developed in the sacrum. We reviewed the medical records and imaging studies of 2 patients diagnosed with DC and the literature published. In the first case, percutaneous biopsy revealed that it was conventional chordoma in the sacrum. Patient underwent radiation therapy (RT). Six years after the RT, the tumor recurred. Surgical removal was performed and the recurrent tumor was diagnosed as DC in histopathologic examination. In the second case, a patient underwent gross total resection of sacral tumor, which was diagnosed with conventional chordoma. Aggravated tumor was detected after 4 months, and patient underwent reoperation. The second operation revealed the transformation of the tumor into DC. The survival time of the patients after the diagnosis was 10 and 31 months. Dedifferentiated chordoma is a rare and highly aggressive tumor. De novo type exists, but it usually transformed from recurrent chordoma after surgical resection or radiation.

Citations

Citations to this article as recorded by  Crossref logo
  • Clival Chordoma Presenting as Airway Obstruction in a Neonate With Tuberous Sclerosis and Good Response to sirolimus
    Aastha Goel, Aakansha Kumari, Sameer Rastogi, Aman Chaudhary, Adarsh Barwad, Atin Kumar
    Pediatric Blood & Cancer.2025;[Epub]     CrossRef
  • De novo Versus Secondary Dedifferentiated Chordomas: A Population-Based Analysis and Integrated Individual Participant Data Meta-Analysis
    Huy Gia Vuong, Minh-Khang Le, Truong P.X. Nguyen, Kathryn Eschbacher
    World Neurosurgery.2023; 173: 208.     CrossRef
  • Chordoma: demographics and survival analysis with a focus on racial disparities and the role of surgery, a U.S. population-based study
    Asad Ullah, Guirshney Samarah Kenol, Kue Tylor Lee, Abdul Qahar Khan Yasinzai, Abdul Waheed, Bina Asif, Imran Khan, Hajra Sharif, Jaffar Khan, Saleh Heneidi, Nabin R. Karki, Tamour Khan Tareen
    Clinical and Translational Oncology.2023; 26(1): 109.     CrossRef
  • Targeted delivery of cancer drug paclitaxel to chordomas tumor cells via an RNA nanoparticle harboring an EGFR aptamer
    Dan Xiao, Yongxiong Huang, Shuaihao Huang, Jianxiong Zhuang, P. Chen, Yi Wang, Lei Zhang
    Colloids and Surfaces B: Biointerfaces.2022; 212: 112366.     CrossRef
  • Chordoma of the Clivus with Metastasis to Femur
    Maruf Matmusaev, Tetsuya Nagatani, Yugo Kishida, Ahmed Ansari
    Asian Journal of Neurosurgery.2022; 17(03): 536.     CrossRef
  • Staging and Classification of Primary Musculoskeletal Bone and Soft-Tissue Tumors According to the 2020 WHO Update, From the AJR Special Series on Cancer Staging
    Mark Douglas Murphey, Mark Jay Kransdorf
    American Journal of Roentgenology.2021; 217(5): 1038.     CrossRef
  • Magnetic Resonance Imaging during Proton Therapy Irradiation Allows for the Early Response Assessment of Pediatric Chordoma
    Sabina Vennarini, Dante Amelio, Stefano Lorentini, Giovanna Stefania Colafati, Antonella Cacchione, Rita De Vito, Andrea Carai, Benedetta Pettorini, Maurizio Amichetti, Angela Mastronuzzi
    Diagnostics.2021; 11(6): 1117.     CrossRef
  • Primary extradural tumors of the spinal column
    Varun Arvind, Edin Nevzati, Maged Ghaly, Mansoor Nasim, Mazda Farshad, Roman Guggenberger, Daniel Sciubba, Alexander Spiessberger
    Journal of Craniovertebral Junction and Spine.2021; 12(4): 336.     CrossRef
  • Treatment of dedifferentiated chordoma: a retrospective study from a large volume cancer center
    Ryan N. Nachwalter, Robert J. Rothrock, Evangelina Katsoulakis, Mrinal M. Gounder, Patrick J. Boland, Mark H. Bilsky, Ilya Laufer, Adam M. Schmitt, Yoshiya Yamada, Daniel S. Higginson
    Journal of Neuro-Oncology.2019; 144(2): 369.     CrossRef
  • State-of-the-Art Imaging in Human Chordoma of the Skull Base
    Rene G. C. Santegoeds, Yasin Temel, Jan C. Beckervordersandforth, Jacobus J. Van Overbeeke, Christianne M. Hoeberigs
    Current Radiology Reports.2018;[Epub]     CrossRef
  • Role of the Apparent Diffusion Coefficient as a Predictor of Tumor Progression in Patients with Chordoma
    T. Sasaki, T. Moritani, A. Belay, A.A. Capizzano, S.P. Sato, Y. Sato, P. Kirby, S. Ishitoya, A. Oya, M. Toda, K. Takahashi
    American Journal of Neuroradiology.2018; 39(7): 1316.     CrossRef
  • Bone and Soft Tissue Pathology
    Julie Gibbs, Evita Henderson-Jackson, Marilyn M. Bui
    Surgical Clinics of North America.2016; 96(5): 915.     CrossRef
  • 9,501 View
  • 72 Download
  • 12 Crossref

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A Chordoma of the Thoracic Spine: A Case Report.
Korean J Spine. 2010;7(4):258-260.
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A Chordoma of the Thoracic Spine: A Case Report.
Korean J Spine. 2010;7(4):258-260.
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A 52-year-old woman presented with a thoracic chordoma at T1-3 level as abnormal sensations on the trunk and low extremities and both legs weakness. Almost total resection was possible through posterolateral costotransversectomy. The patient could ambulate at 3 days after the operation without any sequelae. Histologic study revealed a malignant chordoma with no sarcomatous differentiation.
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  • 36 Download