Chordoma: A rare bone tumor: A single institutional experience on a case series of 23 patients

Abstract

e21519 Background: Chordoma is a rare primary bone tumor arising from primitive notochord remnants of axial skeleton. Adequate surgery still remains the mainstay of its therapeutic treatment. Methods: Our review has been focused on the clinical characteristics, type of treatment, and prognosis of 23 consecutive cases of chordomas observed from 2004 until today and followed by the same team of specialists (orthopedic surgeons and medical oncologists). Results: From June 2004 to December 2008, 23 pts with various stage of disease went to our observation. Male/female: 18/5. Median age at diagnosis: 60 yrs (range: 40 -74). Sites of origin: sacrum (10 pts), spine (12 pts), and skull base (1 pt). Ten pts (44%) had primary disease, 9 pts (39%) had recurrent disease, and 4 pts (17%) had developed metastatic lesions. Surgery was part of the treatment in 21 out of 23 pts. Surgical margins were rated as wide/marginal in 17 pts and intralesional in 4 pts. Adjuvant proton radiotherapy has been delivered to 2 pts. Conventional radiotherapy was performed in 4 pts. The rate of local relapse after primary surgical excision was 76%. The median time to recurrence was 14 months (range: 2–113).Sixteen pts with inoperable/unresectable or metastatic PDGFR β expressing disease were treated with imatinib mesylate (IM) in the context of a clinical trial and a subsequent expanded access program (Stacchiotti S., proc ASCO 2007. abstr. 10003). One pts received weekly cisplatin in combination with IM after progression during IM therapy. The 5-year survival rate of the entire series was 76 %. Two out of 17 pts (12%) surgically treated with adequate margins are still alive and without evidence of disease; two other pts died without evidence of disease. Conclusions: Despite the progress of current surgical techniques and some encouraging results with the use of targeted therapy, chordoma results, generally, in a long-lasting disabling and life-affecting disease. Nevertheless, specific experience of the multidisciplinar team is an important prerequisite to improve patients’ quality of life and, hopefully, outcome. No significant financial relationships to disclose.