Extra-axial chordomas

Abstract

INTRODUCTION: A chordoma is a slow growing malignant tumour of notochordal origin. A tumour with histological features that are identical to those of a chordoma may arise outside the axial skeleton. To date, there is little in the literature documenting their clinical course. METHODS: Our large orthopaedic oncology database was used to document the clinical course of extra-axial chordoma. RESULTS: Over a 30-year period, 131 patients diagnosed with a chordoma were treated at our unit. Only three (2.3%) of these cases were extra-axial chordomas: one in the femur, one in the ulna and one in the proximal fibula. All underwent surgical resection. CONCLUSIONS: In view of the rarity of this tumour and the difficulty in confirming its diagnosis, we suggest that any suspected case is discussed with a specialist sarcoma multidisciplinary team so that the correct diagnosis can be achieved and treatment tailored accordingly.