Preliminary response data, observed by Casali (Cancer, 2004) with imatinib 800 mg/day in
patients affected by chordoma, need to be confirmed by a Phase II study, whose primary
endpoint will be the formal assessment of clinical and pathological response. Aim of the
study will be to explore treatment's activity, but also the potential impact of tumor
response, the feasibility and outcome of subsequent surgery and radiotherapy. In addition,
patterns of tumour response need to be investigated as well, given the peculiar patterns of
response shown with molecular-targeted therapy in solid tumors.