What Are the Conditional Survival and Functional Outcomes After Surgical Treatment of 115 Patients With Sacral Chordoma?

Abstract

Background Conditional survival is a measure of prognosis for patients who have already survived for a specific period of time; however, data on conditional survival after sacrectomy in patients with sacral chordoma are lacking. In addition, because sacral tumors are rare and heterogeneous, classifying them in a way that allows physicians to predict functional outcomes after sacrectomy remains a challenge. Questions/purposes (1) What is the overall survival and disease-free survival in patients treated by sacrectomy for chordoma? (2) What is the conditional survival probability and how do prognostic factors change over time in patients undergoing surgical resection for sacral chordoma? (3) What is the local recurrence rate after surgery, how was it treated, and what factors impact on local recurrence? (4) What is the postoperative motor, sensory, bowel, and bladder function by level of resection as determined by using a newly designed scoring method?Methods Between 2003 and 2012, our center treated 122 patients surgically for sacral chordoma. Of those, two died and five were lost before a minimum followup of 1 year was achieved, leaving 115 patients available for analysis in this retrospective study at a mean of 4.9 years (range, 1.3-10.8 years). Basically, single posterior or combined approaches were chosen based on the most cephalad extent of the tumor and resection level was normally at half or one sacral vertebrae above the tumor. The 5-year conditional survival rate was calculated based on Kaplan-Meier survival analysis. The effect of prognostic factors on conditional survival was also explored. A newly designed score method was proposed and adopted in the current study to critically evaluate the functional outcome after resection of the sacrum. Inter-and intraobserver reliability was tested by a preliminary study using kappa statistics and Spearman rank correlation coefficients. Significant interobserver (p\0.01) and intraobserver agreement (j[0.75) were found in nine items between each observer. Results The estimated 5-year overall survival rate was 81% (95% confidence interval [CI], 72%-90%) at diagnosis. The 5-year disease-free survival rate was 52% (95% CI, 43%-63%). The 5-year conditional overall survival decreased with each additional year in the first 4 years (81% at diagnosis versus 60% at the fourth year, p \ 0.0001) and increased slightly in the fifth year. Patients with adequate surgical margins displayed a higher 5-year survival than those with an inadequate margin (86% [95% CI, 76%-95%] versus 67% [95% CI, 48%-85%], p = 0.01) at diagnosis. Conditional survival estimates for patients who received operations elsewhere were lower than that of newly diagnosed patients treated by us at diagnosis (64% [95% CI, 46%-83%] versus 90% [95% CI, 82%-99%], p = 0.012), but with the numbers we had, we could not detect a difference in conditional survival between those treated elsewhere first compared with those initially treated by us at 5 years. The proposed score system for function evaluation was a...

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

“…Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients. This is consistent to previous studies, and further suggests that an adequate surgical margin is imperative to optimize chordoma treatment and to avoid local progression and recurrence (1,5). Furthermore, we found no correlation between neoadjuvant radiation with prognosis/cyclin E1 expression level.…”

“…As expected, an advanced disease status of chordomas engenders more aggressive future tumor behavior. It is an independent risk factor for OS and MFS in chordoma, which is similar to the results of previous studies ( 2 , 5 , 38 ). Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients.…”

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

“…Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients. This is consistent to previous studies, and further suggests that an adequate surgical margin is imperative to optimize chordoma treatment and to avoid local progression and recurrence (1,5). Furthermore, we found no correlation between neoadjuvant radiation with prognosis/cyclin E1 expression level.…”

“…As expected, an advanced disease status of chordomas engenders more aggressive future tumor behavior. It is an independent risk factor for OS and MFS in chordoma, which is similar to the results of previous studies ( 2 , 5 , 38 ). Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients.…”

Impact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma

“…In our cohort, the recurrence rate was 50% in the R0 group and 67% among patients with positive margins, with a statistically significant association observed in categorical analysis (p = 0.043). These findings are in line with previous larger series: Ruggieri et al reported significantly higher 10-year local recurrence-free survival in patients with wide negative margins (65% vs. 52%, p = 0.045) [ 12 ]; Ji et al identified both tumor size and margin status as significant predictors of 5-year disease-free survival in 115 patients ( p = 0.01) [ 11 ]; and Kiss-Bodolay et al demonstrated superior 5- and 10-year overall survival rates (79% and 59%, respectively) in 101 patients who underwent en bloc resection without capsule violation [ 15 ].…”

“…Specifically, bladder dysfunction was significantly more common in patients with S1–S2 involvement compared to S3–S5 (Fisher’s exact test, p = 0.036). These results are in line with Ji et al (2017), Sharma et al (2021), and Sciubba et al (2009), who emphasized the importance of preserving the S3 nerve roots in maintaining continence [ 8 , 11 , 26 ]. In our series, all patients with S1–S2 involvement developed permanent bladder and anal incontinence, underscoring the critical functional threshold of this anatomical level.…”

Clinical Outcome after Surgical Treatment of Sacral Chordomas: A Single-Center Retrospective Cohort of 27 Patients

“…As for the primary endpoint of our study, the 5-year DSS and 5-year DFS in our cohort were 75% and 54%, respectively. These high relapse rates are indicative of the natural history of these tumors and are consistent with numerous cohort studies of various specialized orthopedic oncology centers, which demonstrate 5-year DSS and 5-year DFS rates of 70–92% and 48–71%, respectively [ 7 , 8 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ] ( Table 3 ). Lee IJ et al demonstrate a 5-year and 10-year DSS of 70% and 47% in a USA-population-based analysis of 473 chordomas of the pelvis [ 20 ], whereas Houdek MT et al report 10-year DSS and 10-year DFS rates of 72% and 58%, respectively, in a large multicenter cohort of 193 patients with sacral chordomas [ 19 ].…”

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

“…Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients. This is consistent to previous studies, and further suggests that an adequate surgical margin is imperative to optimize chordoma treatment and to avoid local progression and recurrence (1,5). Furthermore, we found no correlation between neoadjuvant radiation with prognosis/cyclin E1 expression level.…”

“…As expected, an advanced disease status of chordomas engenders more aggressive future tumor behavior. It is an independent risk factor for OS and MFS in chordoma, which is similar to the results of previous studies ( 2 , 5 , 38 ). Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients.…”

Impact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma

“…In our cohort, the recurrence rate was 50% in the R0 group and 67% among patients with positive margins, with a statistically significant association observed in categorical analysis (p = 0.043). These findings are in line with previous larger series: Ruggieri et al reported significantly higher 10-year local recurrence-free survival in patients with wide negative margins (65% vs. 52%, p = 0.045) [ 12 ]; Ji et al identified both tumor size and margin status as significant predictors of 5-year disease-free survival in 115 patients ( p = 0.01) [ 11 ]; and Kiss-Bodolay et al demonstrated superior 5- and 10-year overall survival rates (79% and 59%, respectively) in 101 patients who underwent en bloc resection without capsule violation [ 15 ].…”

“…Specifically, bladder dysfunction was significantly more common in patients with S1–S2 involvement compared to S3–S5 (Fisher’s exact test, p = 0.036). These results are in line with Ji et al (2017), Sharma et al (2021), and Sciubba et al (2009), who emphasized the importance of preserving the S3 nerve roots in maintaining continence [ 8 , 11 , 26 ]. In our series, all patients with S1–S2 involvement developed permanent bladder and anal incontinence, underscoring the critical functional threshold of this anatomical level.…”

Clinical Outcome after Surgical Treatment of Sacral Chordomas: A Single-Center Retrospective Cohort of 27 Patients

“…As for the primary endpoint of our study, the 5-year DSS and 5-year DFS in our cohort were 75% and 54%, respectively. These high relapse rates are indicative of the natural history of these tumors and are consistent with numerous cohort studies of various specialized orthopedic oncology centers, which demonstrate 5-year DSS and 5-year DFS rates of 70–92% and 48–71%, respectively [ 7 , 8 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ] ( Table 3 ). Lee IJ et al demonstrate a 5-year and 10-year DSS of 70% and 47% in a USA-population-based analysis of 473 chordomas of the pelvis [ 20 ], whereas Houdek MT et al report 10-year DSS and 10-year DFS rates of 72% and 58%, respectively, in a large multicenter cohort of 193 patients with sacral chordomas [ 19 ].…”

Prognostic Significance of Cyclin E1 Expression in Patients With Chordoma: A Clinicopathological and Immunohistochemical Study

“…Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients. This is consistent to previous studies, and further suggests that an adequate surgical margin is imperative to optimize chordoma treatment and to avoid local progression and recurrence (1,5). Furthermore, we found no correlation between neoadjuvant radiation with prognosis/cyclin E1 expression level.…”

“…As expected, an advanced disease status of chordomas engenders more aggressive future tumor behavior. It is an independent risk factor for OS and MFS in chordoma, which is similar to the results of previous studies ( 2 , 5 , 38 ). Moreover, we found a contaminated surgical margin to be an independent risk factor for RFS in chordoma patients.…”

Impact of Surgical Margins and Adjuvant Radiotherapy on Local Recurrence and Survival in Sacral Chordoma

“…In our cohort, the recurrence rate was 50% in the R0 group and 67% among patients with positive margins, with a statistically significant association observed in categorical analysis (p = 0.043). These findings are in line with previous larger series: Ruggieri et al reported significantly higher 10-year local recurrence-free survival in patients with wide negative margins (65% vs. 52%, p = 0.045) [ 12 ]; Ji et al identified both tumor size and margin status as significant predictors of 5-year disease-free survival in 115 patients ( p = 0.01) [ 11 ]; and Kiss-Bodolay et al demonstrated superior 5- and 10-year overall survival rates (79% and 59%, respectively) in 101 patients who underwent en bloc resection without capsule violation [ 15 ].…”

“…Specifically, bladder dysfunction was significantly more common in patients with S1–S2 involvement compared to S3–S5 (Fisher’s exact test, p = 0.036). These results are in line with Ji et al (2017), Sharma et al (2021), and Sciubba et al (2009), who emphasized the importance of preserving the S3 nerve roots in maintaining continence [ 8 , 11 , 26 ]. In our series, all patients with S1–S2 involvement developed permanent bladder and anal incontinence, underscoring the critical functional threshold of this anatomical level.…”

Clinical Outcome after Surgical Treatment of Sacral Chordomas: A Single-Center Retrospective Cohort of 27 Patients

“…As for the primary endpoint of our study, the 5-year DSS and 5-year DFS in our cohort were 75% and 54%, respectively. These high relapse rates are indicative of the natural history of these tumors and are consistent with numerous cohort studies of various specialized orthopedic oncology centers, which demonstrate 5-year DSS and 5-year DFS rates of 70–92% and 48–71%, respectively [ 7 , 8 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 ] ( Table 3 ). Lee IJ et al demonstrate a 5-year and 10-year DSS of 70% and 47% in a USA-population-based analysis of 473 chordomas of the pelvis [ 20 ], whereas Houdek MT et al report 10-year DSS and 10-year DFS rates of 72% and 58%, respectively, in a large multicenter cohort of 193 patients with sacral chordomas [ 19 ].…”