CHORDOMA OF THE SPINE ABOVE THE SACRUM - TREATMENT AND OUTCOME IN 21 CASES

Study Design. Twenty-one cases of chordoma arising in the mobile spine were retrospectively reviewed. Objectives. All the cases were submitt ed to oncologic and surgical staging to correlate treatment and outcom e. Summary of Background Data. Excluding plasmacytomas, chordoma is th e most frequent primary malignant tumor of the spine, occurring mainly in elderly men. The course of the disease is slow, metastases occur l ate, and death can result from complications related to local extensio n of the disease. Complete excision of the tumor according to oncologi c criteria can be hampered by extension of the tumor and by anatomic c onstraints in the mobile spine. Methods. All charts, radiographs, and images were reviewed. The composite information provided by this revie w allowed for oncologic and surgical staging of these cases. Treatment was defined according to Ennekings criteria. All the patients were fo llowed for determination of their status clinically and radiographical ly Results. Ten patients died (1 to 137 months after treatment, mean 6 5 months); four patients are alive with the disease; only seven patien ts (33%) are symptom free at the final follow-up (39 to 112 months aft er treatment, mean 65 months). Conventional radiation therapy was not effective in eradicating the tumor, even if associated with palliative or debulking surgery: of 15 cases, 12 were associated with recurrence of progression. Intralesional surgery also was not effective (two rec urrences in two cases, 18 to 41 months later). En bloc excision of the lesion, sometimes combined with radiation therapy as an adjuvant, obt ained the best results (four patients disease free at 39 to 112 months , mean 77 months). Conclusions. En bloc excision-even if marginal-is t he treatment of choice of chordomas of the spine. Early diagnosis and careful surgical staging and planning are necessary. Megavoltage radia tion can be administered as an adjuvant.