Chordoma. A clinicopathologic and prognostic study of a Swedish national series

Abstract

Chordoma is a rare tumor with slow growth developing from remnants of the notochord and thus appears in close relation to the axial skeleton. A Swedish national series collected over a period of 13 years, comprising 51 patients with a follow-up time of 8-20 years, was studied. Histologically all tumors had a typical appearance except two, which had characteristics of "chondroid chordoma". Fifty-seven per cent of the tumors were located in the sacrococcygeal region, 27 per cent in the spheno-occipital region and 16 per cent in the vertebrae. The peak age incidence was in the 6th and 7th decades. Male: female ratio was 1:1. The main symptoms were pain and neurologic disturbances. Skeletal destruction was noted radiographically in most instances. Intratumoral calcifications were rarely seen. The treatment was surgery, radiotherapy or a combination of these. There was only one long-term survivor without evidence of disease, a patient operated on for cervical chordoma 14 years earlier. Six other patients lived 8-18 years after diagnosis with chordoma. All other patients were dead; 39 died of chordoma, five of unrelated causes. Distant metastases were noted in 29 per cent. Chordoma constituted 17.5 per cent of all primary malignant bone tumors of the axial skeleton. The yearly incidence was 0.51 per million inhabitants in Sweden.