Exploring pharmacotherapeutic options for treating chordoma

Chordoma is a rare, primary bone cancer arising along the midline from the skull base to the sacrum. Chordomas appear histologically low-grade but are highly invasive and often recur locally. Management is maximal surgical resection. Radiation therapy using protons and photons is often necessary because complete resection is frequently not possible due to tumor invasion into surrounding critical structures. Cytotoxic chemotherapy is often ineffective. The high rate of recurrence is reflected by a median survival of 6-7 years. The authors present a review of the current and emerging medical treatments for chordoma and the rationale supporting their use.