Department of Neurosurgery, Johns Hopkins University School of Medicine, 600 N. Wolfe St., Meyer 5-185A, Baltimore, MD 21287, USA.
Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA.
Spine J. 2021 Mar;21(3):500-517. doi: 10.1016/j.spinee.2020.10.009. Epub 2020 Oct 10.
Chordoma is a notochord-derived primary tumor of the skull base and vertebral column known to affect 0.08 to 0.5 per 100,000 persons worldwide. Patients commonly present with mechanical, midline pain with or without radicular features secondary to nerve root compression. Management of these lesions has classically revolved around oncologic resection, defined by en bloc resection of the lesion with negative margins as this was found to significantly improve both local control and overall survival. With advancement in radiation modalities, namely the increased availability of focused photon therapy and proton beam radiation, high-dose (>50 Gy) neoadjuvant or adjuvant radiotherapy is also becoming a standard of care. At present chemotherapy does not appear to have a role, but ongoing investigations into the ontogeny and molecular pathophysiology of chordoma promise to identify therapeutic targets that may further alter this paradigm. In this narrative review we describe the epidemiology, histopathology, diagnosis, and treatment of chordoma.
脊索瘤是一种源自颅底和脊柱的脊索源性原发性肿瘤，据世界范围内统计，其发病率为每 10 万人中有 0.08 至 0.5 人。患者常表现为机械性、中线疼痛，伴有或不伴有神经根受压引起的根性特征。这些病变的传统治疗方法围绕着肿瘤学切除术，即整块切除病变并保证阴性切缘，因为这被发现显著提高了局部控制率和总体生存率。随着放射治疗模式的进步，即聚焦光子治疗和质子束放射治疗的可用性增加，高剂量（>50Gy）新辅助或辅助放疗也成为一种标准治疗方法。目前化疗似乎没有作用，但对脊索瘤的发生和分子病理生理学的持续研究有望确定治疗靶点，这可能进一步改变这一治疗模式。在这篇叙述性综述中，我们描述了脊索瘤的流行病学、组织病理学、诊断和治疗。
