Department of Neurosurgery, The University of Texas M.D. Anderson Cancer Center, 1515 Holcombe Blvd, Unit 442, Houston, TX, 77030-4009, USA.
Curr Treat Options Oncol. 2021 Mar 20;22(5):40. doi: 10.1007/s11864-021-00838-z.
Management of chordoma along the cranial-spinal axis is a major challenge for both skull base and spinal surgeons. Although chordoma remains a rare tumor, occurring in approximately 1 per 1 million individuals, its treatment poses several challenges. These tumors are generally poorly responsive to radiation and chemotherapy, leading to surgical resection as the mainstay of treatment. Due to anatomic constraints and unique challenges associated with each primary site of disease, gross total resection is often not feasible and is associated with high rates of morbidity. Additionally, chordoma is associated with high rates of recurrence due to the tumor's aggressive biologic features, and postoperative radiation is increasingly incorporated as a treatment option for these patients. Despite these challenges, modern-day surgical techniques in both skull base and spinal surgery have facilitated improved patient outcomes. For example, endoscopic endonasal techniques have become the mainstay in resection of skull base chordomas, improving the ability to achieve gross total resection, while reducing associated morbidity of open transfacial techniques. Resection of spinal chordomas has been facilitated by emerging techniques in preoperative imaging, intraoperative navigation, spinal reconstruction, and radiotherapy. Taken collectively, the treatment of chordoma affecting the skull base and spinal requires a multidisciplinary team of surgeons, radiation oncologists, and medical oncologists who specialize in the treatment of this challenging disease.
颅脊柱轴内脊索瘤的治疗是颅底和脊柱外科医生面临的重大挑战。尽管脊索瘤仍然是一种罕见的肿瘤，发病率约为每 100 万人中有 1 例，但它的治疗存在多种挑战。这些肿瘤通常对放疗和化疗反应不佳，导致手术切除成为主要治疗方法。由于解剖限制和每个病变原发部位的独特挑战，往往无法实现肿瘤全切除，且与高发病率相关。此外，由于肿瘤具有侵袭性的生物学特征，脊索瘤的复发率较高，术后放疗越来越多地被纳入这些患者的治疗选择。尽管存在这些挑战，但颅底和脊柱外科的现代手术技术已经促进了患者预后的改善。例如，内镜经鼻技术已成为颅底脊索瘤切除的主要方法，提高了实现肿瘤全切除的能力，同时降低了开放式经面技术相关的发病率。术前影像学、术中导航、脊柱重建和放疗等新兴技术促进了脊柱脊索瘤的切除。总的来说，颅底和脊柱脊索瘤的治疗需要由擅长治疗这种具有挑战性疾病的外科医生、放射肿瘤学家和肿瘤内科医生组成的多学科团队来完成。
