Snyderman Carl H, Gardner Paul A
Department of Otolaryngology.
Center for Cranial Base Surgery, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
Curr Opin Otolaryngol Head Neck Surg. 2020 Apr;28(2):118-121. doi: 10.1097/MOO.0000000000000614.
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.
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.
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.
由于其位置和浸润性行为，斜坡区脊索瘤是具有挑战性的肿瘤。治疗的主要目标仍然是全切除（GTR）。手术技术的进步，尤其是鼻内镜技术的应用，提高了手术切除率，同时将手术并发症降至最低。
斜坡脊索瘤治疗的进展包括鼻内镜入路至斜坡和岩斜区的进一步发展，对脊索瘤生物学行为和分子标志物预后价值的深入理解，以及对质子束治疗等新型放射治疗方式的经验增加。
改进的手术技术提高了我们实现全切除同时将手术发病率降至最低的能力。分子标志物可将患者分层为不同预后组，并有助于指导进一步治疗。手术全切除与质子束或调强放疗相结合目前提供了最佳的治愈潜力。新型免疫系统调节剂治疗前景广阔，但仅用于临床试验。
