Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research. A total of 42 patients underwent 85 surgeries; median follow-up was 15.8 years, overall survival rate was 49.9% at 10 years; meanwhile, progression-free survival was 26.6% at 10 years. A significantly improved survival was observed in younger patients (<50 years), in tumors with Ki67 ≤ 5% and when adjuvant radiotherapy was performed. To conclude, clival chordomas are aggressive tumors in which surgery and radiotherapy play a fundamental role while molecular targeted drugs still have an ancillary position. Recognizing risk factors for recurrence and performing a molecular characterization of more aggressive lesions may be the key to future effective treatment.
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http://dx.doi.org/10.3390/cancers15184493 | DOI Listing |
Brain Spine
November 2024
Department of Neurosurgery, Heidelberg University Hospital, Heidelberg, Germany.
N Am Spine Soc J
December 2024
Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States.
Background: Chordomas are rare, slow growing, locally aggressive malignant bone tumors that arise from remnants of the embryonic notochord with variable presenting symptoms depending on tumor location.
Methods: All patients with craniospinal chordoma managed at our institution between 1982 and 2023 were retrospectively reviewed. Demographics, tumor characteristics, clinical course and treatment, and long-term neurological and survival outcomes were collected.
Int J Mol Sci
November 2024
Department of Neurosurgery, University of Colorado, Anschutz Medical Campus, Aurora, CO 80045, USA.
Chordomas are rare, generally slow-growing spinal tumors that nonetheless exhibit progressive characteristics over time, leading to malignant phenotypes and high recurrence rates, despite maximal therapeutic interventions. The tumors are notoriously resistant to therapies and are often located in regions that complicate achieving gross total resections. Cell lines from these tumors are rare as well.
View Article and Find Full Text PDFJ Neurosurg Case Lessons
November 2024
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota.
Background: The authors applied laser interstitial thermal therapy (LITT) to a recurrent skull base chordoma, which has not been previously described.
Observations: A 63-year-old man was initially diagnosed with an 8-cm destructive clival chordoma, which was aggressively resected endoscopically but recurred despite multiple operations, proton radiation therapy, and chemotherapy. The patient underwent uncomplicated LITT for a subtemporal mass, which palliated the tumor for 10 months.
Front Oncol
October 2024
Department of Neurosurgery, General Hospital of Northern Theater Command, Shenyang, Liaoning, China.
Introduction: The surgery of clival chordoma remains one of the most formidable challenges for neurosurgeons because of its location at great depth in the cranium and proximity to critical neurovascular structures. Here, we describe the technique and feasibility of the purely endoscopic far-lateral supracerebellar infratentorial approach (EF-SCITA) for resection of an intradural clival chordoma.
Case Description: A 68-year-old women presented with sudden ptosis on the left side for two weeks.
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