Metastatic chordoma with pancreatic disease and response to imatinib

Introduction: Chordomas are rare tumours arising from embryological remnants of the notochord, consisting of 1-4% of bone neoplasms. Despite their low grade character, they frequently recur and management can be limited by anatomical location and minimal systemic therapy options. We report a case of recurrent chordoma with an unusual pattern of metastases, subsequent managed with imatinib. Method: A 45 year old lady presented with a neck lump. Imaging demonstrated a C3-C6 prevertebral mass with pathology inkeeping with chordoma. Following surgical resection and C3-C5 vetebrectomy, the patient underwent proton beam therapy. 2 years following resection, local recurrence occurred with metastases in the pancreatic neck. Imatinib, a novel therapy in chordoma, was commenced. In the early stage of her imatinib therapy she developed spinal cord compression, which responded well to palliative radiotherapy. Results: Her disease remains stable on imatinib as demonstrated on serial imaging. She is tolerating treatment reasonably well with minimal toxicity. Conclusion: While metastases in chordoma are common, affecting up to 40% of patients, lesions are typically found in skin, bone or the lungs. Identification of a metastasis in the pancreas, mark this case as displaying an extremely unusual pattern of disease. This case also adds to evidence for the potential utility of imatinib in recurrent chordoma, given documented disease stabilisation, symptomatic improvement and low toxicity profile. This provides further rationale for investigation of imatinib as a targeted therapy in larger patient cohorts, vital as there is no systemic therapy available.