What differential diagnoses should be considered?
Differential diagnoses to be considered include Ewing sarcoma, chondrosarcoma, giant cell tumour, metastases and lymphoma.
At what age do chordomas usually present?
Chordomas are usually seen in adults (usually 30 – 60 years). Less than 5% present in children.
Which cell type seen on microscopy confirms the diagnosis of chordoma?
Physaliphorous cell (these cells appear vacuolated because they contain large intracytoplasmic droplets of mucoid material).
Where are chordomas typically located?
Chordomas may be found anywhere along the craniospinal axis: sacro-coccygeal (approximately 50%), spheno-occipital (approximately 35%), vertebral body (approximately 15%).
MRI demonstrates an extradural soft tissue mass which involves the basiocciput, anterior arch of C1 and C2 vertebral body. The mass is intermediate to low signal on T1WI, high signal on T2WI and enhances on the post-contrast images. It causes significant mass effect, with compression of the cord at the cervicomedullary junction.