Sacral Chordoma: Long-Term Outcome of A Large Series of Patients Surgically Treated At Two Reference Centers

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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 due to local failure. To date, few large series with long follow up are available in literature. METHODS.: all consecutive patients(pts) 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.: 99 pts were identified: 65 male,34 female. Median age was 59 years (range 22–77), median tumor size 9?cm (range 4–22).19 pts received pre/post-operative radiotherapy(RT). Wide(R0) surgical margins were achieved in 46 pts, marginal(R1) margins in 43 and intralesional(R2) margins in 10. At a median follow-up of 8.7 years (range 1–23.8) 30 pts died of disease,31 pts developed local relapse,16 pts distant metastasis while 51 pts 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. 15-yrs DFS was, in fact, 49% for R0 and 7% for R1 respectively. CONCLUSIONS.: in this series, long-term outcome of resected sacral chordoma was poor, with less than 25% pts disease-free at 15 years. Interestingly, only half of pts 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

Original languageEnglish
Pages (from-to)1049-1057
Number of pages9
Publication statusAccepted/In press - Apr 6 2016

ASJC Scopus subject areas

  • Clinical Neurology
  • Orthopedics and Sports Medicine


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