Study design: Retrospective case series.
Objective: To report on the natural history and long-term outcome of a large series of consecutive primary sacral chordoma patients surgically treated at two reference centers.
Summary of background data: Sacral chordomas are rare tumors with poor long-term prognosis mainly caused by local failure. Till date, a few large series with long follow up are available in literature.
Methods: All consecutive patients affected by primary localized sacral chordoma operated on at two Italian reference centers between 1981 and 2012 were included. Overall survival (OS), disease free survival (DFS), crude cumulative incidence (CCI) of local recurrence (LR), and distant metastases (DM) were calculated. Multivariable analyses for OS, DFS, LR, and DM were performed.
Results: A total of 99 patients were identified: 65 males and 34 females. Median age was 59 years (range 22-77 yrs), median tumor size was 9 cm (range 4-22). Nineteen patients received pre- or postoperative radiotherapy (RT). Wide (R0) surgical margins were achieved in 46 patients, marginal (R1) margins in 43 patients and intralesional (R2) margins in 10 patients. At a median follow up of 8.7 years (range 1-23.8 yrs) 30 patients died of disease, 31 patients developed local relapse, 16 patients developed distant metastasis, whereas 51 patients are alive without disease. OS and DFS at 5, 10, and 15 year were 92% and 63%, 45% and 62%, 36% and 21%, respectively, without any evidence of a plateau in the curves.CCI of LR and DM were 30% and 9% at 5 years, 46% and 18% at 10 years, 56% and 23% at 15 years. Size of the tumor and quality of surgical margins were the only significant predictors of long-term outcome. DFS for 15 years was, in fact, 49% for R0 and 7% for R1, respectively.
Conclusion: In this series, long-term outcome of resected sacral chordoma was poor, with less than 25% patients were disease-free at 15 years. Interestingly, only half of the patients treated with R0 resection had no evidence of recurrence at 15 years. When surgical margins are expected to be positive other treatment modalities should be considered, especially when expected sequelae are substantial as in the case of more cephalad levels of resection.
Level of evidence: 3.