Mobile spine chordoma: results of 166 patients from the AOSpine Knowledge Forum Tumor database

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>A chordoma is an indolent primary spinal tumor that has devastating effects on the patient's life. These lesions are chemoresistant, resistant to conventional radiotherapy, and moderately sensitive to proton therapy; however, en bloc resection remains the preferred treatment for optimizing patient outcomes. While multiple small and largely retrospective studies have investigated the outcomes following en bloc resection of chordomas in the sacrum, there have been few large-scale studies on patients with chordomas of the mobile spine. The goal of this study was to review the outcomes of surgically treated patients with mobile spine chordomas at multiple international centers with respect to local recurrence and survival. This multiinstitutional retrospective study collected data between 1988 and 2012 about prognosis-predicting factors, including various clinical characteristics and surgical techniques for mobile spine chordoma. Tumors were classified according to the Enneking principles and analyzed in 2 treatment cohorts: Enneking-appropriate (EA) and Enneking-inappropriate (EI) cohorts. Patients were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation; otherwise, they were categorized as EI.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>Descriptive statistics were used to summarize the data (Student t-test, chi-square, and Fisher exact tests). Recurrence and survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and multivariate Cox proportional hazard modeling.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>A total of 166 patients (55 female and 111 male patients) with mobile spine chordoma were included. The median patient follow-up was 2.6 years (range 1 day to 22.5 years). Fifty-eight (41%) patients were EA and 84 (59%) patients were EI. The type of biopsy (p &lt; 0.001), spinal location (p = 0.018), and if the patient received adjuvant therapy (p &lt; 0.001) were significantly different between the 2 cohorts. Overall, 58 (35%) patients developed local recurrence and 57 (34%) patients died. Median survival was 7.0 years postoperative: 8.4 years postoperative for EA patients and 6.4 years postoperative for EI patients (p = 0.023). The multivariate analysis showed that the EI cohort was significantly associated with an increased risk of local recurrence in comparison with the EA cohort (HR 7.02; 95% CI 2.96–16.6; p &lt; 0.001), although no significant difference in survival was observed.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>EA resection plays a major role in decreasing the risk for local recurrence in patients with chordoma of the mobile spine.</jats:p></jats:sec>

Prognostic factors in spinal chordoma: An update of current systematic review and meta‐analysis

“…We commend the authors for performing this interesting study as these helpful results would be useful to make a balanced treatment decision. However, we noted that the authors did not find extent of tumor invasion or worse preoperative Frankel score to be an independent predictor, which contradicts the results from previous studies …”

“…However, we noted that the authors did not find extent of tumor invasion or worse preoperative Frankel score to be an independent predictor, which contradicts the results from previous studies. [2][3][4][5] To date, an increasing number of clinical and histopathologic factors as well as molecular biomarkers have been investigated for their association with spinal chordoma prognosis, 6 but the results are still inconclusive or controversial. We previously performed a systematic review on prognostic factors in spinal chordoma, 6 but no definitive conclusions could be derived due to heterogeneity and the scarcity of included studies.…”

Role of the Apparent Diffusion Coefficient as a Predictor of Tumor Progression in Patients with Chordoma

“…Surgical resection is the standard therapy for chordoma, though there have been recent advances in radiation therapy (RT). [6][7][8][9][10][11] En bloc surgical resection with negative margins and no intraoperative spill is associated with a reduced rate of local recurrence. However, the location of the chordoma may limit the ability to perform a gross total resection.…”

Spinal stereotactic body radiotherapy following intralesional curettage with separation surgery for initial or salvage chordoma treatment

“…3,4,6,7,9,13,17,20,25,27,34,39,[42][43][44]49,55 While en bloc resection is the treatment of choice for chordoma, not all chordomas are conducive to en bloc resection to achieve marginal or wide margins due to their relationship to other vital structures such as the spinal cord or vertebral arteries. 23,31 However, some authors have argued that aggressive resection is indicated even in the face of severe neurological sacrifice. With up to 32% of reported chordomas arising in the mobile spine, there remains a need to explore alternatives to traditional en bloc treatment in such complex cases with unique anatomical constraints.…”