두개저 척삭종에 대한 치료 전략

Management Strategy for Chordomas of the Skull Base

Chordomas occur rarely in the skull base but are remaining challenge with respect to the management. The aim of this study was to investigate the clinical results in an institutional series of this tumor and analyze the treatment outcome. Between 1991 and 2005, nineteen consecutive patients with pathological diagnosis of chordoma of the skull base were managed by multimodal treatment combining surgical resection with conventional photon radiotherapy and/or gamma knife radiosurgery (GKS). A retrospective analysis was conducted on these patients (aged 3 years to 69 years; mean age of 36.9 years; 15 females and 4 males; mean follow-up of 56.0 months). Total or near-total resection was achieved in 3 patients (15.8%), subtotal was done in 11 (57.9%), and partial in 5 (26.3%). Adjuvant radiotherapy and GKS were performed on 13 and 4 patients, respectively. Four deaths occurred during the follow-up: 2 resulted from progression of the disease and another 2 from other causes. The progressionfree survival at 3 years and 5 years was 61.5% and 40.0%. Among 15 survivors, 4 patients were suffering from severe disability with progressive disease. As the biological behavior of chordoma is such an aggressive one in the skull base, initial aggressive management should be directed combining maximum safe resection with high-dose radiotherapy such as proton beam radiation or radiosurgery.