Chordomas are rare but locally aggressive cancer, which originate from primitive notochord remnants. Guidelines have recently been proposed to include the option of choosing chordomas in different locations. However, there is still a great challenge in the modern management of chordomas, primarily due to the high recurrence rate and poor prognosis. On this basis, there is a high demand for new therapeutic approaches and reliable prognostic factors. Recent progress in studying the molecular basis of this specific type of cancer has deepened the understanding of this mechanism, which overall facilitates the discovery of specific biomarkers or indicators of the disease. It also gives rise to potential targeted therapies against chordomas as evidenced by the fact that some RTK inhibitors in a clinical context have been evaluated in relation to chordomas. This article summarizes these achievements including the studies relative to pathogenesis, prognostic factors, and targeted therapies for chordomas. The theme of existing problems is also mentioned, which would facilitate general future efforts in this field.

中文翻译：

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脊索瘤简述：发病机理，预后因素和治疗靶点。

脊索瘤是罕见的但局部侵袭性癌症，其起源于原始脊索脊残余。最近提出了一些指南，其中包括在不同位置选择脊索瘤的选择。然而，由于高复发率和不良预后，脊索瘤的现代管理仍面临巨大挑战。在此基础上，对新的治疗方法和可靠的预后因素有很高的要求。研究这种特定类型癌症的分子基础的最新进展加深了对该机制的理解，这总体上促进了该疾病特定生物标志物或指标的发现。它也引起了针对脊索瘤的潜在靶向疗法，这一事实证明了一些RTK抑制剂已在临床背景下针对脊索瘤进行了评估。本文总结了这些成就，包括有关脊索瘤的发病机理，预后因素和靶向疗法的研究。还提到了现有问题的主题，这将促进该领域今后的总体努力。