Object: Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon's ability to achieve radical tumor resection are also evaluated.
Methods: Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average follow-up was 66 months (median 60 months, range 3-189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation.
Results: Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications.
Conclusions: Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.
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http://dx.doi.org/10.3171/2009.9.JNS08596 | 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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