Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature
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...
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Elsevier
2024-12-01
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| Series: | North American Spine Society Journal |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666548424002622 |
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| author | Sarah Lee Nahom Teferi Juan Vivanco-Suarez Ajmain Chowdhury Stephen Glennon Kyle Kato Tyson Matern Kathryn L. Eschbacher Michael Petronek Patrick Hitchon |
| author_facet | Sarah Lee Nahom Teferi Juan Vivanco-Suarez Ajmain Chowdhury Stephen Glennon Kyle Kato Tyson Matern Kathryn L. Eschbacher Michael Petronek Patrick Hitchon |
| author_sort | Sarah Lee |
| collection | DOAJ |
| description | 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. Adjuvant radiotherapy (RT) was stratified into standard dose fractionated radiotherapy (standard XRT) for doses of 50 to 60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60 to 81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed. Results: A total of 37 patients were included in our cohort (mean age 46.0 ± 20.8 years; 22 male). Clival chordomas accounted for the majority of patients (56.8%), followed by vertebral (27%) and sacral (10.8%) chordomas. Thirty-five patients (94.6%) underwent gross total resection (GTR) or subtotal resection (STR), and 2 patients underwent excisional biopsy only. Postoperatively, functional status trended towards improvement (KPS: Preop- 80 [range 40–100] vs. Post op- 90 [60–100], p = .0911) and all patients either maintained or improved their neurological function. Median overall survival (OS) after diagnosis was 16.5 years. Age < 65, clival tumor location, post-operative Frankel grade E, and administration of adjuvant RT following initial STR significantly improved OS. OS of GTR patients was not significantly affected by adjuvant RT treatment. Conclusions: Our results show the best long-term survival outcomes for chordoma patients undergoing GTR of tumor tissue. Higher postoperative neurological function was significantly associated with OS, highlighting the importance of maximal but safe total tumor resection. Moreover, adjuvant RT improved long-term survival for patients that underwent STR but had no effect on survival outcomes for patients that underwent GTR. |
| format | Article |
| id | doaj-art-080cf9d1dd334fe884e888135c59ae2c |
| institution | OA Journals |
| issn | 2666-5484 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | Elsevier |
| record_format | Article |
| series | North American Spine Society Journal |
| spelling | doaj-art-080cf9d1dd334fe884e888135c59ae2c2025-08-20T01:57:52ZengElsevierNorth American Spine Society Journal2666-54842024-12-012010056910.1016/j.xnsj.2024.100569Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literatureSarah Lee0Nahom Teferi1Juan Vivanco-Suarez2Ajmain Chowdhury3Stephen Glennon4Kyle Kato5Tyson Matern6Kathryn L. Eschbacher7Michael Petronek8Patrick Hitchon9Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United States; Corresponding author. Department of Neurosurgery, University of Iowa Hospitals and Clinics 200 Hawkins Dr., Iowa City, IA, United States, 52242.Department of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesUniversity of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesUniversity of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Pathology, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Radiation Oncology, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesDepartment of Neurosurgery, University of Iowa Carver, College of Medicine, Iowa City, IA, United StatesBackground: 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. Adjuvant radiotherapy (RT) was stratified into standard dose fractionated radiotherapy (standard XRT) for doses of 50 to 60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60 to 81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed. Results: A total of 37 patients were included in our cohort (mean age 46.0 ± 20.8 years; 22 male). Clival chordomas accounted for the majority of patients (56.8%), followed by vertebral (27%) and sacral (10.8%) chordomas. Thirty-five patients (94.6%) underwent gross total resection (GTR) or subtotal resection (STR), and 2 patients underwent excisional biopsy only. Postoperatively, functional status trended towards improvement (KPS: Preop- 80 [range 40–100] vs. Post op- 90 [60–100], p = .0911) and all patients either maintained or improved their neurological function. Median overall survival (OS) after diagnosis was 16.5 years. Age < 65, clival tumor location, post-operative Frankel grade E, and administration of adjuvant RT following initial STR significantly improved OS. OS of GTR patients was not significantly affected by adjuvant RT treatment. Conclusions: Our results show the best long-term survival outcomes for chordoma patients undergoing GTR of tumor tissue. Higher postoperative neurological function was significantly associated with OS, highlighting the importance of maximal but safe total tumor resection. Moreover, adjuvant RT improved long-term survival for patients that underwent STR but had no effect on survival outcomes for patients that underwent GTR.http://www.sciencedirect.com/science/article/pii/S2666548424002622ClivalSpinalSacralChordomaRadiotherapyProton beam therapy |
| spellingShingle | Sarah Lee Nahom Teferi Juan Vivanco-Suarez Ajmain Chowdhury Stephen Glennon Kyle Kato Tyson Matern Kathryn L. Eschbacher Michael Petronek Patrick Hitchon Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature North American Spine Society Journal Clival Spinal Sacral Chordoma Radiotherapy Proton beam therapy |
| title | Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature |
| title_full | Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature |
| title_fullStr | Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature |
| title_full_unstemmed | Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature |
| title_short | Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature |
| title_sort | surgical management of skull base and spinal chordomas a case series with comprehensive review of the literature |
| topic | Clival Spinal Sacral Chordoma Radiotherapy Proton beam therapy |
| url | http://www.sciencedirect.com/science/article/pii/S2666548424002622 |
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