Abstract
Chordomas are slowly growing, locally aggressive primary malignant neoplasms derived from primitive notochordal cells. They tend to occur at the extreme of ages and at the two ends of the spinal column. The purpose of this study was to evaluate the presentation, morphology and behaviour of 20 non-clival, non-sacral chordoma cases.
Details of 20 cases of histologically confirmed Chordomas of the mobile spine (Cervical-7, Thoracic-7, Lumbar-6) between 1967 and 2006, were extracted from the Scottish Bone Tumour Registry. The casenotes and radiographs were retrospectively reviewed.
Non-sacral chordomas comprised 48.7% (20/41 cases) of total chordomas registered. There was a slight feminine predominance (11/20 cases). Mean age was 58.1 years (range, 12 to 82 years). Progressively worsening back pain was the first symptom in all the patients. Seven had intra-lesional (five recurred), six marginal (1 recurred) and 2 wide resections (none recurred). Eleven of the twenty-one patients were treated with adjuvant radiation therapy. In five patients, the chordoma was inoperable and all but one were treated with radiotherapy. There were 2 recurrences in the group of patients treated without adjuvant radiotherapy.
Six patients (40%) developed local recurrences and 3 patients (15%) developed metastases. There were 4 survivors including one with local recurrence. Sixteen patients died including 3 with metastases, 7 with persistent primary disease, 1 with local recurrence and 5 with no evidence of disease or died of unrelated causes. Median survival was 19 months (mean-30.2 months), with 5- and 10-year survival rates 10% and 0.0% respectively.
We found that non-sacral/non-clival chordomas had a much poorer prognosis than published in the literature. Local recurrences occurred in 40%. Intra-lesional resection should be avoided as it is associated with 71.4% local recurrence in our series.
