The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study

Abstract

Objective: Chordoma, a rare malignant tumor originating from embryonal notochord remnants, exhibits high resistance to conventional treatments, making surgical resection imperative. However, the factors influencing prognosis specifically for cervical spine chordoma have not been clearly identified. We investigate the prognosis of cervical spine chordoma with factors influential in a nationwide multicenter retrospective study.

Methods: This study included all patients diagnosed with cervical spine chordoma at 7 tertiary referral centers from January 1998 to March 2023, excluding those with clivus and thoracic spine chordomas extending into the cervical spine. Local recurrence (LR) was identified through follow-up magnetic resonance imaging, either as reappearance in completely resected tumors or regrowth in residual tumors. The study assessed LR and overall survival, analyzing factors influencing LR and death.

Results: Forty-five patients with cervical spine chordoma had a mean age of 46.4 years. Over a median follow-up of 52 months, LR and distant metastasis were observed in 21 (46.7%) and 4 patients (8.9%), respectively, and 16 patients (36%) were confirmed dead. The 5-year and 10-year cumulative LR rates were 51.3% and 60%, respectively, while the 5-year and 10-year survival rates were 82% and 53%. Age was the only significant factor affecting mortality (hazard ratio, 1.04; 95% confidence interval, 1.04-1.07; p=0.015). Notably, the degree of resection and adjuvant therapy did not statistically significantly impact local tumor control and mortality.

Conclusion: This study, the largest multicenter retrospective analysis of cervical spine chordoma in Korea, identified age as the only factor significantly affecting patient survival.

Keywords: Cervical spine; Chordoma; Recurrence; Surgery; Survival rate; Treatment outcome.

Fig. 1.

The distribution of cervical spine chordomas. (A) The distribution of vertebral body involvement in cervical spine chordoma is as follows (per total number of vertebral involvement, 106): C2 involvement was the most common, observed in 34 patients (32.1%, 34 out of 106), followed by C3 involvement in 27 patients (25.5%, 27 out of 106). C1 involvement was noted in 15 patients (14.2%), while C4 involvement was seen in 12 patients (11.3%). Involvement of C5 was observed in 6 patients (5.7%). Both C6 and C7 showed involvement in 5 patients each (4.7%). Additionally, C0 and T1 involvement were each observed in 1 patient (0.9%). C0 and T1 level chordomas were cases where cervical spine chordoma extended to the C0 and T1 levels, respectively. (B) The distribution of epicenter for cervical spine chordomas is as follows (per total number of patients, 45): C2 was the most common, observed in 30 patients (66.7%), followed by C3 in 5 patients (11.1%). C4 and C5 were involved in 3 patients (6.7%) each, while C6 was involved in 2 patients (4.4%). C1 and C7 were the epicenter in 1 patient (2.2%) each.

Fig. 2.

Schematic overview of the treatment and outcomes for 45 cervical spine chordomas. Among a total of 45 patients, 3 underwent biopsy, 2 of whom were lost to follow-up (FU), and the remaining one received proton therapy (PT). The other 42 patients underwent operation (OP). One patient received neoadjuvant therapy (Neoadj) and OP, but experienced a recurrence, leading to a secondary OP and adjuvant therapy (adj). Following another recurrence and the detection of distant metastasis, the patient underwent stereotactic radiosurgery (SRS) and targeted therapy (TT). Of the 11 patients who underwent OP alone, 2 were lost to FU, and recurrence occurred in 6 patients. One of the patients who experienced a recurrence was subsequently lost to FU. One patient experienced a recurrence after secondary OP and subsequently underwent tertiary OP. Four patients received secondary OP and adj; of these, 2 experienced further recurrences, 1 was lost to FU, and another underwent tertiary OP and adj. Thirty patients received OP and adj, with 14 experiencing recurrences; 3 of these were lost to FU. Two patients received radiation therapy (RT), 1 of whom experienced a recurrence and was subsequently lost to FU. Five patients underwent secondary OP; of these, one had a recurrence and received tertiary OP, while another, diagnosed with distant metastasis, underwent RT, PT, and chemotherapy (CT). Four patients underwent secondary OP and adj, all of whom experienced recurrences. One patient underwent tertiary OP following a recurrence and subsequently required quaternary OP. Another patient received RT and, after a recurrence, underwent tertiary OP. Recurrences and distant metastasis were confirmed in 2 patients: one received RT and SRS, and the other underwent tertiary OP and SRS, followed by recurrence and additional SRS. Bx, biopsy; SD, stable disease; OS, overall survival; PD, progressive disease.

Fig. 3.

Cumulative incidence of local recurrence (LR) and overall survival of cervical spine chordomas. (A) The cumulative incidence of LR among 42 patients with cervical spine chordoma was reported as follows: the 5-year cumulative LR rate was 51.3% (95% confidence interval [CI], 32.8%–67.1%), and the 10-year cumulative LR rate was 60% (95% CI, 34.7%–78.2%). (B) The overall survival (OS) after initial surgery for 41 patients with cervical spine chordoma was as follows: the 5-year OS rate was 82% (95% CI, 64.1%–91.8%), and the 10-year OS rate was 53% (95% CI, 30.5%–71.3%). (C) Comparing OS after initial surgery between patients aged 46 years and older and those under 46 years old (p<0.005). The median survival for patients under 46 years was 81.6 months, while the median survival for those aged 46 years and older was 51.6 months. (D) Comparing OS after initial surgery in patients 20 years of age or younger and those 60 years of age or older (p=0.039). The median survival for patients 20 years or younger was 81.6 months, while the median survival for those 60 years or older was 46.8 months.