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Chordoma: Natural History and Results in 28 Patients Treated at a Single Institution

From the Departments of Surgery (DB, AG, EP, MF, MS), Radiation Oncology (LL), and Pathology (MC), National Cancer Institute of Milan, Italy.

Correspondence: Address correspondence and reprint requests to: Alessandro Gronchi, MD, Department of Surgery, National Cancer Institute of Milan, Via Venezian 1, 20133, Milano, Italy; Fax: 39-02-23902404; E-mail: alessandro.gronchi{at}istitutotumori.mi.it

Background: The natural history of chordoma is characterized by a high failure rate and a poor functional outcome. The purpose of this study was to review the long-term outcome of our institutional experience.

Methods: The clinical features, type of treatment, pathologic assessment, and follow-up of 56 consecutive patients with chordoma were reviewed.

Results: Fifty sacral and six mobile spine chordomas (median size, 13 cm; range, 2–30 cm) were treated at our center between January 1933 and December 2000. Twenty-eight patients affected by sacrococcygeal chordoma and operated on after 1977 form the basis of our study. Surgical margins were rated as wide in 11 cases, marginal in 13 cases, and intralesional in 4 cases. The median follow-up was 71 months (range, 15–200 months). Seventeen patients’ disease recurred. Ten patients died as a result of disease. Nine patients remained continuously free of disease. The estimated 5- and 10-year overall survival was, respectively, 87.8% and 48.9%; disease-free survival was 60.6% and 24.2%. Radiotherapy was considered for marginal and intralesional resections.

Conclusions: High sacral amputation can achieve a good rate of wide-margin resections for sacrococcygeal chordomas. Adjuvant radiotherapy may offset the negative effect in the prognosis of marginal resections.

Key Words: Chordoma • Sarcoma • Sacrococcygeal tumors • Prognosis • Surgery • Radiotherapy

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