Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas

In this study, we evaluated patients' clinical outcome and recurrence rates at long-term follow-up after aggressive microsurgical resection of cranial base chordomas.

Seventy-four patients with chordomas underwent operations during a 16-year period from 1988 to 2004. The philosophy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for remnants. Staged operations were performed for extensive tumors or if a sizable tumor remnant was noted after the first resection. Patients included primary (previously untreated) and previously operated or irradiated cases. Information was prospectively gathered concerning the patients' neurological condition, Karnofsky Performance Scale score, and tumor status on magnetic resonance imaging scans.

There were 47 primarily operated patients (63.5%) and 27 patients (36.5%) who had previously undergone surgery or radiotherapy. A total of 121 procedures were performed in 74 patients. The mean follow-up period was 96 months, with a range of 1 to 198 months. A single stage removal was performed in 41 (55.4%) of the patients and multiple stage removal was performed in 33 (44.5%) of the patients. Gross total removal was accomplished in 53 (71.6%) of the patients, and subtotal resection was accomplished in 21 (28.4%) of the patients. During the follow-up period, 24 (32%) of the patients had no evidence of disease, 37 (50%) of the patients were alive with evidence of disease, 11 (14.8%) of the patients died of disease, and two (2.7%) of the patients died of complications. Recurrence-free survival at 10 years was 31% for the whole group, 42% for the primarily operated patients, and 26% for the reoperation cases (P = 0.0001). The average Karnofsky Performance Scale score was 80 +/- 11.7 preoperatively, 84 +/- 8.9 at the 1-year follow-up, and 86 +/- 12.8 at the last follow-up in surviving patients. No conclusion could be drawn regarding the value of radiotherapy because of the treatment philosophy and the small number of patients.

Aggressive microsurgical resection of chordomas can be followed by long-term, tumor-free survival with good functional outcome. A more conservative strategy is recommended in reoperation cases, especially after previous radiotherapy, to reduce postoperative complications.