Chordomas and Chondrosarcomas of the Posterior Fossa

Skull base chordomas and chondrosarcomas are rare bone neoplasms that develop around the clivus and the petroclival region. Although they are quite similar in terms of clinical and radiological features, chordomas and chondrosarcomas differ with regard to their origin and prognosis. The optimal treatment for skull base chordomas and high-grade chondrosarcomas includes radical surgical resection followed by high-dose radiotherapy. Because of chordomas and chondrosarcomas rarity and complex location, the surgical management of these lesions should be carried out exclusively by expert skull base teams. Providing a unique trajectory to the clivus, the endoscopic endonasal approach has considerably changed the surgical management of posterior fossa chordomas and chondrosarcomas in the last two decades. However, limitations remain, and transcranial approaches remain the best adjunct for complex lesions extending beyond the limits of what can be safely achieved with endoscopic techniques. Endoscopically assisted transcranial approaches have also made possible to widen the exposure of classic transcranial approach and to reduce the aggressiveness of the approach. For chordomas, radical resection, including infiltrated bone, remains the goal, as it is the most important prognostic factor. Skull base chondrosarcomas carry a more favorable outcome than chordomas with a better long-term control. Conversely, despite aggressive treatment, chordomas have a high rate of recurrence. The development of medical targeted therapies is strongly needed to improve the outcome of patients with advanced disease.