Clinical Reviews

Purpose. Our purpose was to discuss the optimal treatment and outcomes for patients with skull base chordomas. Methods. We reviewed the pertinent literature for this study. Results. Skull base chordomas usually arise in the clivus and are rarely completely resectable. Therefore, most are treated with radiotherapy (RT). Because of the risk of severe late complications, the dose is often limited with conventional photon RT, and the probability of cure is low. Proton RT alone or combined with photon RT (proton/photon RT) offers the advantage of improved dose distribution and the ability to treat the tumor to a higher dose without exceeding normal tissue tolerance. The 10-year local control rate after proton/photon RT is approximately 40% to 50%. The probability of local control is related to minimum tumor dose and dose inhomogeneity. Conclusions. Skull base chordoma is a rare neoplasm that is rarely cured after surgery alone or combined with conventional RT. Proton/photon RT offers the advantage of increasing the tumor dose while minimizing the dose to normal tissues, thus reducing the risk of late complications. The optimal treatment may be photon/proton RT alone or combined with a gross total resection, when feasible. A 2005 Wiley Periodicals, Inc. Head Neck 27: 159–165, 2005