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Surgical Management of Skull base and Spinal Chordomas: A Case Series with Comprehensive Review of the Literature
Journal article   Open access   Peer reviewed

Surgical Management of Skull base and Spinal Chordomas: A Case Series with Comprehensive Review of the Literature

Sarah Lee, Nahom Teferi, Juan Vivanco-Suarez, A J Chowdhury, Stephen Glennon, Kyle Kato, Tyson Matern, Kathryn L. Eschbacher, Michael Petronek and Patrick Hitchon
North American Spine Society journal (NASSJ), Vol.20, 100569
12/2024
DOI: 10.1016/j.xnsj.2024.100569
PMCID: PMC11648787
PMID: 39687058
url
https://doi.org/10.1016/j.xnsj.2024.100569View
Published (Version of record) Open Access

Abstract

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. 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 50-60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60-81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed. 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. 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.
 Radiotherapy Chordoma Clival Proton beam therapy Sacral Spinal

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