Demographic and clinicopathologic characteristics of chordoma patients
According to the inclusion and exclusion criteria in Fig. 1, our study included a total of 1,080 eligible chordoma patients from 1975 to 2017. The number of married group, divorced/separated group, widowed group and single group was 700 (64.8%), 88 (8.1%), 78 (7.2%) and 214 (19.8%), respectively. Table 1 shows the clinical characteristics and demographic of all adult chordoma patients. Chi-square test showed that marital status was related to diagnosis year (p = 0.014), age at diagnosis (p < 0.001), sex (p < 0.001), primary site (p = 0.019) and surgery (p < 0.001). With the increase of years, the proportion of chordoma patients also increased. In the whole cohort, the majority of patients were male (59.9%), the primary site was bones of skull and face and associated joints (40.4%), and the localized stage (40.9%). In addition, the proportion of widowed patients over 60 years old (94.9%), female (73.1%) and surgery not performed (37.2%) was higher than other three groups.
Table 1
Baseline demographic and clinical characteristics of chordoma patients in our study.
Characteristic | Total No. (%) | Married | | Divorced/Separated | | Widowed | | Single | P value |
No. (%) | | No. (%) | | No. (%) | | No. (%) |
Total | 1080 | 700 (64.8) | | 88 (8.1) | | 78 (7.2) | | 214 (19.8) | |
Year of diagnosis | | | | | | | | | 0.014 |
1975–1988 | 98 (9.1) | 70 (10.0) | | 4 (4.5) | | 12 (15.4) | | 12 (5.6) | |
1988–2002 | 262 (24.3) | 181 (25.9) | | 19 (21.6) | | 20 (25.6) | | 42 (19.6) | |
2003–2016 | 720 (66.7) | 449 (64.1) | | 65 (73.9) | | 46 (59.0) | | 160 (74.8) | |
Age at diagnosis | | | | | | | | | < 0.001 |
< 40 | 242 (22.4) | 131 (18.7) | | 18 (20.5) | | 0 (0.0) | | 93 (43.5) | |
40–60 | 412 (38.1) | 294 (42.0) | | 35 (39.8) | | 4 (5.1) | | 79 (36.9) | |
> 60 | 426 (39.4) | 275 (39.3) | | 35 (39.8) | | 74 (94.9) | | 42 (19.6) | |
Sex | | | | | | | | | < 0.001 |
Male | 647 (59.9) | 441 (63.4) | | 46 (52.3) | | 21 (26.9) | | 136 (63.6) | |
Female | 433 (40.1) | 256 (36.6) | | 42 (47.7) | | 57 (73.1) | | 78 (36.4) | |
Primary site | | | | | | | | | 0.019 |
Bones of skull and face and associated joints | 436 (40.4) | 285 (40.7) | | 34 (38.6) | | 17 (21.8) | | 100 (46.7) | |
Vertebral column | 279 (25.8) | 179 (25.6) | | 25 (28.4) | | 27 (34.6) | | 48 (22.4) | |
Pelvic bone, sacrum, coccyx and associated joints | 365 (33.8) | 236 (33.7) | | 29 (33.0) | | 34 (43.6) | | 66 (30.8) | |
Disease stage | | | | | | | | | 0.587 |
Localized | 442 (40.9) | 288 (41.1) | | 38 (43.2) | | 30 (38.5) | | 86 (40.2) | |
Regional | 454 (42.0) | 290 (41.4) | | 41 (46.6) | | 32 (41.0) | | 91 (42.5) | |
Distant | 90 (8.3) | 55 (7.9) | | 5 (5.7) | | 7 (9.0) | | 23 (10.7) | |
Unstaged | 94 (8.7) | 67 (9.6) | | 4 (4.5) | | 9 (11.5) | | 14 (6.5) | |
Tumor size | | | | | | | | | 0.084 |
<5 cm | 357 (33.1) | 229 (32.7) | | 32 (36.4) | | 18 (23.1) | | 78 (36.4) | |
5-10cm | 256 (23.7) | 155 (22.1) | | 21 (23.9) | | 21 (26.9) | | 59 (27.6) | |
> 10 cm | 101 (9.4) | 64 (9.1) | | 10 (11.4) | | 5 (6.4) | | 22 (10.3) | |
Unknown | 366 (33.9) | 252 (36.0) | | 25 (28.4) | | 34 (43.6) | | 55 (25.7) | |
Histological type | | | | | | | | | 0.953 |
Conventional chordoma | 1019 (94.4) | 659 (94.1) | | 83 (94.3) | | 75 (96.2) | | 202 (94.4) | |
Chondroid chordoma | 54 (5.0) | 37 (5.3) | | 4 (4.5) | | 3 (3.8) | | 10 (4.7) | |
Dedifferentiated chordoma | 7 (0.6) | 4 (0.6) | | 1 (1.1) | | 0 (0.0) | | 2 (0.9) | |
Surgery | | | | | | | | | < 0.001 |
Surgery not performed | 177 (16.4) | 99 (14.1) | | 20 (22.7) | | 29 (37.2) | | 29 (13.6) | |
STR | 452 (41.9) | 295 (42.1) | | 38 (43.2) | | 23 (29.5) | | 96 (44.9) | |
GTR | 301 (27.9) | 199 (28.4) | | 23 (26.1) | | 12 (15.4) | | 67 (31.3) | |
Unknown extent of resection | 150 (13.9) | 107 (15.3) | | 7 (8.0) | | 14 (17.9) | | 22 (10.3) | |
Radiotherapy | | | | | | | | | 0.734 |
Yes | 544 (50.4) | 354 (50.6) | | 48 (54.5) | | 40 (51.3) | | 101 (47.7) | |
No | 536 (49.6) | 346 (49.4) | | 40 (45.5) | | 38 (48.7) | | 112 (52.3) | |
Chemotherapy | | | | | | | | | 0.639 |
Yes | 40 (3.7) | 24 (3.4) | | 3 (3.4) | | 2 (2.6) | | 11 (5.1) | |
No | 1040 (96.3) | 676 (96.6) | | 85 (96.6) | | 76 (97.4) | | 203 (94.9) | |
Note: P-value < 0.05 are shown in bold. |
Abbreviations: STR, subtotal resection; GTR, gross total/radical resection. |
Percentages may not total 100 because of rounding. |
Survival Of Patients With Chordoma
By analyzing the Kaplan-Meier curve with a log-rank test, we found that age at diagnosis (p < 0.001), marital status (p < 0.001), primary site (p < 0.001), disease stage (p < 0.001), tumor size (p < 0.001), histological type (p = 0.002), surgery (p < 0.001) and chemotherapy (p = 0.001) were associated with OS (Table 2). The 5-year OS and 10-year OS of married, divorced/separated, widowed and single patients were 73.7% and 51.5%, 69.5% and 42.8%, 45.2% and 12.5%, 75.6% and 57.0%, respectively, and the median survival time of married, divorced/separated, widowed and single patients was 125.0 months, 103.0 months, 56.0 months and 157.0 months, respectively (Fig. 2). Widowed patients had the lowest 5-year OS, 10-year OS and median overall survival time, while single patients had the highest 5-year OS, 10-year OS and median overall survival time. After stratifying the primary site and disease stage, we still observed similar results (Table 3 and Fig. 3).
Table 2
Kaplan–Meier analysis overall survival for chordoma patients.
Characteristic | 5-year Overall Survival, % | 10-year Overall Survival, % | Median Overall Survival (months) | Kaplan-Meier |
Log Rank χ2 test | P value |
Age at diagnosis | | | | 164.433 | < 0.001 |
< 40 | 83.8 | 74.4 | - | | |
40–60 | 82.8 | 58.7 | 138.0 | | |
> 60 | 54.3 | 25.2 | 68.0 | | |
Sex | | | | 2.585 | 0.108 |
Male | 70.5 | 45.9 | 105.0 | | |
Female | 72.9 | 52.0 | 132.0 | | |
Marital status | | | | 66.240 | < 0.001 |
Married | 73.7 | 51.5 | 125.0 | | |
Divorced/Separated | 69.5 | 42.8 | 103.0 | | |
Widowed | 45.2 | 12.5 | 56.0 | | |
Single | 75.6 | 57.0 | 157.0 | | |
Primary site | | | | 41.055 | < 0.001 |
Bones of skull and face and associated joints | 79.2 | 65.1 | 253.0 | | |
Vertebral column | 66.7 | 37.9 | 90.0 | | |
Pelvic bone, sacrum, coccyx and associated joints | 66.4 | 40.1 | 91.0 | | |
Disease stage | | | | 29.554 | < 0.001 |
Localized | 77.6 | 55.3 | 147.0 | | |
Regional | 71.6 | 48.2 | 105.0 | | |
Distant | 49.7 | 32.7 | 53.0 | | |
Unstaged | 65.2 | 39.0 | 89.0 | | |
Tumor size | | | | 45.181 | < 0.001 |
< 5 cm | 82.3 | 70.9 | 243.0 | | |
5-10cm | 70.5 | 45.9 | 106.0 | | |
> 10 cm | 55.9 | 32.1 | 70.0 | | |
Unknown | 67.0 | 41.0 | 94.0 | | |
Histological type | | | | 12.265 | 0.002 |
Conventional chordoma | 71.5 | 47.6 | 110.0 | | |
Chondroid chordoma | 76.6 | 72.8 | - | | |
Dedifferentiated chordoma | 28.6 | 28.6 | 14.0 | | |
Surgery | | | | 97.790 | < 0.001 |
Surgery not performed | 48.3 | 26.0 | 56.0 | | |
STR | 78.1 | 58.5 | 154.0 | | |
GTR | 82.9 | 59.3 | 178.0 | | |
Unknown extent of resection | 59.1 | 31.9 | 80.0 | | |
Radiotherapy | | | | 0.140 | 0.708 |
Yes | 72.7 | 47.1 | 106.0 | | |
No | 70.0 | 49.7 | 120.0 | | |
Chemotherapy | | | | 11.445 | 0.001 |
Yes | 47.9 | 28.1 | 51.0 | | |
No | 72.4 | 49.4 | 119.0 | | |
Note: P-value < 0.05 are shown in bold. |
Abbreviations: STR, subtotal resection; GTR, gross total/radical resection. |
Table 3
Kaplan–Meier analysis overall survival for chordoma patients based on primary site and disease stage.
Characteristic | 5-year Overall Survival, % | 10-year Overall Survival, % | Median Overall Survival (months) | Kaplan-Meier |
Log Rank χ2 test | P value |
Primary site | | | | 35.462 | < 0.001 |
Bones of skull and face and associated joints | | | | | |
Married | 81.0 | 68.3 | 253.0 | | |
Divorced/Separated | 65.8 | 44.9 | 106.0 | | |
Widowed | 45.3 | 18.1 | 42.0 | | |
Single | 85.0 | 71.8 | - | | |
Vertebral column | | | | 38.754 | < 0.001 |
Married | 68.2 | 38.9 | 97.0 | | |
Divorced/Separated | 82.8 | 40.9 | 102.0 | | |
Widowed | 32.5 | 0.0 | 33.0 | | |
Single | 74.1 | 58.3 | 141.0 | | |
Pelvic bone, sacrum, coccyx and associated joints | | | | 7.278 | 0.064 |
Married | 69.6 | 44.9 | 106.0 | | |
Divorced/Separated | 62.6 | 45.0 | 81.0 | | |
Widowed | 54.7 | 19.6 | 63.0 | | |
Single | 63.1 | 32.4 | 80.0 | | |
Disease stage | | | | | |
Localized | | | | 33.341 | < 0.001 |
Married | 78.1 | 57.8 | 166.0 | | |
Divorced/Separated | 78.5 | 58.5 | 146.0 | | |
Widowed | 47.0 | 10.4 | 59.0 | | |
Single | 87.2 | 65.8 | 178.0 | | |
Regional | | | | 31.648 | < 0.001 |
Married | 74.4 | 50.3 | 121.0 | | |
Divorced/Separated | 68.4 | 40.7 | 87.0 | | |
Widowed | 40.0 | 13.3 | 46.0 | | |
Single | 76.5 | 59.7 | - | | |
Distant | | | | 17.771 | < 0.001 |
Married | 59.4 | 39.9 | 80.0 | | |
Divorced/Separated | 0.0 | 0.0 | 12.0 | | |
Widowed | 28.6 | 0.0 | 16.0 | | |
Single | 42.3 | 30.2 | 32.0 | | |
Note: P-value < 0.05 are shown in bold. |
Identification Of Prognostic Factors Of Chordoma Patients OS
Univariate and multivariate Cox regression were used to analyze the prognostic factors associated with OS of chordoma patients (Table 4). Univariate Cox regression analysis showed that age at diagnosis, marital status, primary site, disease stage, tumor size, histological type, surgery and chemotherapy were related factors (all p < 0.05) of OS in chordoma patients (Fig. 4). Moreover, after the all factors were included in multivariate analysis, primary site, histological type, radiotherapy and chemotherapy were not independent risk factors for patients with chordoma (Fig. 5). In addition, multivariate analysis showed that widowed patients had the worst OS (HR: 1.71; 95% CI: 1.25–2.33, p < 0.001) compared with married patients.
Table 4
Univariate and multivariate analysis of overall survival rates.
Characteristic | Univariate analysis | Multivariate analysis |
Hazard Ratio (95% CI) | P value | Hazard Ratio (95% CI) | P value |
Age at diagnosis | | < 0.001 | | < 0.001 |
< 40 | Reference | | Reference | |
40–60 | 1.92 (1.40–2.64) | < 0.001 | 1.97 (1.42–2.73) | < 0.001 |
> 60 | 4.83 (3.57–6.53) | < 0.001 | 4.28 (3.08–5.96) | < 0.001 |
Sex | | | | |
Male | Reference | | Reference | |
Female | 0.86 (0.71–1.04) | 0.109 | 0.82 (0.67-1.00) | 0.048 |
Marital status | | < 0.001 | | 0.006 |
Married | Reference | | Reference | |
Divorced/Separated | 1.29 (0.92–1.80) | 0.136 | 1.42 (1.01–1.99) | 0.046 |
Widowed | 2.82 (2.13–3.73) | < 0.001 | 1.71 (1.25–2.33) | < 0.001 |
Single | 0.83 (0.64–1.09) | 0.175 | 1.16 (0.88–1.53) | 0.303 |
Primary site | | < 0.001 | | 0.181 |
Bones of skull and face and associated joints | Reference | | Reference | |
Vertebral column | 1.93 (1.52–2.44) | < 0.001 | 1.19 (0.92–1.54) | 0.196 |
Pelvic bone, sacrum, coccyx and associated joints | 1.94 (1.54–2.44) | < 0.001 | 0.97 (0.73–1.28) | 0.809 |
Disease stage | | < 0.001 | | < 0.001 |
Localized | Reference | | Reference | |
Regional | 1.41 (1.13–1.75) | 0.002 | 1.43 (1.15–1.79) | 0.002 |
Distant | 2.22 (1.62–3.05) | < 0.001 | 2.31 (1.66–3.20) | < 0.001 |
Unstaged | 1.68 (1.24–2.29) | 0.001 | 1.17 (0.85–1.62) | 0.327 |
Tumor size | | < 0.001 | | < 0.001 |
< 5 cm | Reference | | Reference | |
5-10cm | 1.96 (1.47–2.61) | < 0.001 | 1.25 (0.91–1.71) | 0.173 |
> 10 cm | 2.98 (2.08–4.27) | < 0.001 | 1.85 (1.22–2.80) | 0.004 |
Unknown | 2.04 (1.57–2.65) | < 0.001 | 1.50 (1.13–1.98) | 0.005 |
Histological type | | 0.004 | | 0.124 |
Conventional chordoma | Reference | | Reference | |
Chondroid chordoma | 0.52 (0.29–0.92) | 0.025 | 0.78 (0.43–1.42) | 0.419 |
Dedifferentiated chordoma | 3.03 (1.25–7.32) | 0.014 | 2.11 (0.83–5.35) | 0.115 |
Surgery | | < 0.001 | | < 0.001 |
Surgery not performed | Reference | | Reference | |
STR | 0.39 (0.30–0.52) | < 0.001 | 0.58 (0.44–0.76) | < 0.001 |
GTR | 0.35 (0.26–0.46) | < 0.001 | 0.41 (0.30–0.55) | < 0.001 |
Unknown extent of resection | 0.82 (0.63–1.07) | 0.144 | 0.95 (0.71–1.26) | 0.723 |
Radiotherapy | | | | |
Yes | Reference | | Reference | |
No | 0.97 (0.80–1.16) | 0.709 | 1.07 (0.89–1.30) | 0.466 |
Chemotherapy | | | | |
Yes | Reference | | Reference | |
No | 0.51 (0.34–0.76) | 0.001 | 0.71 (0.47–1.09) | 0.120 |
Abbreviations: STR, subtotal resection; GTR, gross total/radical resection. |
In the stratification of primary site and disease stages (Table 5), univariate analysis showed that marital status was a risk factor for OS in the primary site of “Bones of skull and face and associated joints”, “Vertebral column”, and “Localized”, “Regional”, and “Distant” disease stages. In addition, multivariate analysis showed that marital status was an independent risk factor for the primary site of “Bones of skull and face and associated joints” and “Vertebral column”. Moreover, although marital status was not an independent risk factor for the prognosis of chordoma patients at the disease stages of “Localized”, “Regional”, and “Distant”, widowed patients were at higher risk of survival compared with married, divorced/separated or single patients.
Table 5
Univariate and multivariate analysis of overall survival rates based on primary site and disease stage.
Characteristic | Univariate analysis | Multivariate analysis |
Hazard Ratio (95% CI) | P value | Hazard Ratio (95% CI) | P value |
Primary site | | | | |
Bones of skull and face and associated joints | | < 0.001 | | 0.017 |
Married | Reference | | Reference | |
Divorced/Separated | 2.01 (1.14–3.56) | 0.016 | 2.31 (1.26–4.22) | 0.007 |
Widowed | 4.33 (2.35-8.00) | < 0.001 | 2.34 (1.18–4.65) | 0.015 |
Single | 0.74 (0.45–1.21) | 0.233 | 1.15 (0.67–1.95) | 0.618 |
Vertebral column | | < 0.001 | | 0.011 |
Married | Reference | | Reference | |
Divorced/Separated | 0.81 (0.44–1.51) | 0.514 | 0.94 (0.48–1.86) | 0.863 |
Widowed | 3.33 (2.11–5.26) | < 0.001 | 2.43 (1.44–4.12) | 0.001 |
Single | 0.66 (0.39–1.10) | 0.111 | 0.82 (0.48–1.43) | 0.488 |
Pelvic bone, sacrum, coccyx and associated joints | | 0.064 | | 0.277 |
Married | Reference | | Reference | |
Divorced/Separated | 1.26 (0.72–2.19) | 0.423 | 1.24 (0.69–2.25) | 0.477 |
Widowed | 1.74 (1.12–2.71) | 0.014 | 0.96 (0.57–1.60) | 0.867 |
Single | 1.36 (0.90–2.05) | 0.140 | 1.54 (0.99–2.38) | 0.056 |
Disease stage | | | | |
Localized | | < 0.001 | | 0.483 |
Married | Reference | | Reference | |
Divorced/Separated | 1.15 (0.62–2.17) | 0.655 | 1.17 (0.60–2.29) | 0.650 |
Widowed | 3.54 (2.16–5.80) | < 0.001 | 1.54 (0.89–2.67) | 0.126 |
Single | 0.79 (0.48–1.29) | 0.340 | 1.20 (0.70–2.06) | 0.519 |
Regional | | < 0.001 | | 0.055 |
Married | Reference | | Reference | |
Divorced/Separated | 1.22 (0.77–1.93) | 0.401 | 1.31 (0.81–2.11) | 0.266 |
Widowed | 2.80 (1.82–4.32) | < 0.001 | 1.95 (1.23–3.10) | 0.005 |
Single | 0.69 (0.45–1.04) | 0.073 | 1.09 (0.71–1.67) | 0.702 |
Distant | | < 0.001 | | 0.159 |
Married | Reference | | Reference | |
Divorced/Separated | 6.83 (2.25–20.73) | 0.001 | 4.43 (1.19–16.53) | 0.027 |
Widowed | 2.78 (1.14–6.80) | 0.025 | 3.14 (0.71–13.99) | 0.133 |
Single | 1.31 (0.69–2.48) | 0.405 | 1.33 (0.58–3.04) | 0.497 |