A retrospective clinicopathological study of 37 patients with chordoma: a danish national series.

Purpose. There are, in general, few published series on chordoma. It is a rare disease and further data are still needed.Patients/methods. The data of 37 patients with chordoma were retrospectively analyzed. Treatment was surgical excision in 11, radical radiotherapy in 9 and a combination of the two in 16 cases. The male to female ratio was 2.7 : 1. Median age was 59 years (range 1-89 years).Results. The most common symptoms at diagnosis were pain (98%), neurological disturbances (42%) and incontinence (33%). The tumours were located in the sacro-coccygeal region in 68%, the spheno-occipital region in 16% and the vertebrae in 16% of the patients. Median tumour.size was 7 cm (range 1-30 cm). Local recurrence occurred in 21/36 treated cases and distant metastases developed in eight patients (23%). The median time to recurrence/progression after primary treatment was 2 years (range 1-10 years). The actuarial 5-year rates of overall, progression-free and symptom-free survival were 40%, 31% and 20%, respectively. The corresponding 10-year rates were 26%, 21% and 14%, respectively. At the time of analysis, seven patients were alive, six without evidence of disease. Four of the six patients without active disease were symptom free. A univariate analysis showed that age, sex, tumour size, histopathology, surgical safety margin, treatment modality and radiation dose did not significantly affect overall, progression-free or symptom-free survival. Only turnout site had a prognostic value with turnouts in the spheno-occipital region carrying the worst prognosis.Discussion. We conclude that effective treatment against chordomas is still lacking and a prospective multi-institutional registration study may provide more information on the optimal work-up and treatment of this rare disease.