ABSTRACT
Chordomas are difficult and challenger tumors to treat. Despite of its pathological appearance that demonstrates an slow-growing behavior, their location and patterns of spread through the base of the skull make these tumors one of the most difficult diseases to treat at the base of the skull. The inadequate response to the conventional radiation therapy and the high incidence of recurrence, in cases treated with classical neurosurgical approaches, make mandatory a radical removal of the tumor mass and the surrounding pathological bone which can only be achieved with the use of skull base approaches associated with an extensive drilling of the bony tumor. In order to reach the goal of the surgical treatment of the skull base chordomas, the mastery of the skull base anatomy and the management of the drill are crucial points to obtain a successful results. In this report, we express our philosophy to treat these tumors which is based on the radical removal associated to a postoperative proton beam therapy. The main characteristic of the skull base chordomas is to spread through the base of the skull, which dictates the surgical approach that should be employed for each patient. In this article we will address the management of the skull base chordomas, with special emphasis to the role of the surgical and radiation treatment. The pathologic diagnosis and the radiological findings of chordomas of the skull base also will be discussed.