Golding Regina, Abuqubo Rami, Pansa Christopher J, Bhatta Manish, Shankar Vishal, Mani Kyle, Kleinbart Emily, Gelfand Yaroslav, Murthy Saikiran, De la Garza Ramos Rafael, Krystal Jonathan, Eleswarapu Ananth, Yassari Reza, Mostafa Evan, Fourman Mitchell S, Schlumprecht Anne
Department of Orthopaedic Surgery, Montefiore Einstein, Bronx, NY 10461, USA.
Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
J Clin Med. 2024 Sep 24;13(19):5679. doi: 10.3390/jcm13195679.
Chordomas are rare sarcomas arising from notochordal tissue and occur most commonly in the spine. The standard of care for chordomas without evidence of metastatic disease generally consists of en bloc resection followed by adjuvant radiotherapy. However, long-term (20-year) survival rates are approximately 30%. Chordomas are generally considered as chemo resistant. Therefore, systemic therapies have rarely been employed. Novel immunotherapies, including antibody therapy and tumor vaccines, have shown promise in early trials, leading to extended progression-free survival and symptom relief. However, the outcomes of larger trials using these vectors are heterogeneous. The aim of this review is to summarize novel chordoma treatments in immune-targeted therapies. The current merits, trial outcomes, and toxicities of these novel immune and targeted therapies, including those targeting vascular endothelial growth factor receptor (VEGFR) targets and the epidermal growth factor receptor (EGFR), will be discussed.
脊索瘤是一种起源于脊索组织的罕见肉瘤，最常见于脊柱。对于无转移疾病证据的脊索瘤，标准治疗方案通常包括整块切除，随后进行辅助放疗。然而，长期（20年）生存率约为30%。脊索瘤通常被认为对化疗耐药。因此，很少采用全身治疗。包括抗体治疗和肿瘤疫苗在内的新型免疫疗法在早期试验中显示出了前景，可延长无进展生存期并缓解症状。然而，使用这些载体的更大规模试验的结果并不一致。本综述的目的是总结免疫靶向治疗中脊索瘤的新型治疗方法。将讨论这些新型免疫和靶向治疗的当前优点、试验结果和毒性，包括那些针对血管内皮生长因子受体（VEGFR）靶点和表皮生长因子受体（EGFR）的治疗。
