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.