Department of Surgery, Aga Khan University Hospital, Karachi Pakistan.
J Pak Med Assoc. 2024 Sep;74(9):1718-1720. doi: 10.47391/JPMA.24-72.
Chordomas are rare malignant neoplasms arising from vestigial remnants of the embryonic notochord. Approximately 55-70% of chordomas develop within the vertebral column. Their affinity to develop within the bones of the axial skeleton and propensity to locally invade and recur makes them challenging candidates for complete surgical excision. Adjuvant therapies are hence necessary to improve outcomes; for which chemotherapy has been observed to be largely ineffective, owing to the tumour being resistant to it. Radiotherapy is the current adjuvant therapy of choice for chordoma management. Over the years, proton beam therapy (PBT) has been the subject of medical attention, given the dosimetric benefits it confers over traditional radiotherapy, allowing more concentrated radiation to be given to the target of interest and reducing damage to surrounding normal tissue. A review of the current literature reveals PBT offers significantly better outcomes when used as an adjuvant to maximal surgical resection rather than as a definitive therapy.
脊索瘤是一种罕见的恶性肿瘤,起源于胚胎脊索的残余物。大约 55-70%的脊索瘤发生在脊柱内。它们在轴骨骼的骨骼内发育的亲和力以及局部侵袭和复发的倾向,使它们成为完全手术切除的具有挑战性的候选者。因此,需要辅助治疗来改善结果;由于肿瘤对其具有耐药性,因此观察到化疗效果不大。放射治疗是目前治疗脊索瘤的辅助疗法。多年来,质子束治疗(PBT)一直受到医学关注,因为它相对于传统放射治疗具有剂量学优势,允许向感兴趣的目标给予更集中的辐射,并减少对周围正常组织的损伤。对当前文献的回顾表明,与作为确定性治疗相比,PBT 作为最大程度的手术切除的辅助治疗时提供了显著更好的结果。