Metastatic chordoma with pancreatic disease and response to imatinib

A 45-year-old woman presented with a left-sided neck swelling following treatment a year prior for cervical spine chordoma. She had initially been managed surgically with a cervical vertebrectomy and a course of proton beam therapy. Although there had been a degree of residual tissue, her disease remained stable radiologically and clinically. Repeat MRI demonstrated an increasing left paravertebral mass and a head of pancreas metastasis, which shared pathological characteristics with chordoma. Given the advanced metastatic nature of her disease, imatinib was offered with a palliative intent. While waiting for treatment she developed a spinal cord compression, managed with radiotherapy. She commenced imatinib and her disease remained stable for 9 months before progressing clinically and radiologically. This case demonstrates an unusual pattern of metastatic chordoma and provides further rationale for imatinib in such patients.