Current opinion in otolaryngology and head and neck surgery: clival chordoma and its management.

PURPOSE OF REVIEW

Chordomas of the clival region are challenging tumors to treat due to their location and infiltrative behavior. The primary goal of treatment remains a gross total resection (GTR). Advances in surgical techniques, especially the adoption of endoscopic endonasal techniques, provide improved rates of surgical resection while minimizing secondary surgical morbidities.

RECENT FINDINGS

Advances in the management of clival chordomas include further development of endoscopic endonasal approaches to the clivus and petroclival region, enhanced understanding of the biologic behavior of chordomas and prognostic value of molecular markers, and increased experience with newer radiation therapy modalities such as proton beam therapy.

SUMMARY

Improved surgical techniques enhance our ability to achieve a GTR while minimizing morbidity of surgery. Molecular markers may allow stratification of patients into prognostic groups and help direct further therapy. A combination of surgery with GTR and proton beam or intensity modulated radiotherapy currently offers the best potential for cure. Therapy with new immune system modulators is promising but is reserved for clinical trials.