Division of Translational Radiation Sciences, Department of Radiation Oncology, University of Maryland, School of Medicine, Baltimore, MD 21201, USA.
Department of Cell Biology and Molecular Genetics, University of Maryland, College Park, MD 20742, USA.
Curr Cancer Drug Targets. 2022 Aug 15;22(8):678-690. doi: 10.2174/1568009622666220419122716.
Chordoma is a rare, slow-growing sarcoma that is locally aggressive and typically resistant to conventional chemo- and radiotherapies. Despite its low incidence, chordoma remains a clinical challenge because therapeutic options for chordoma are limited, and little is known about the molecular mechanisms involved in resistance to therapies. Furthermore, there are currently no established predictive or prognostic biomarkers to follow disease progression or treatment. Whole-genome sequencing of chordoma tissues has demonstrated a low-frequency mutation rate compared to other cancers. This has generated interest in the role of epigenetic events in chordoma pathogenesis. In this review, we discuss the current understanding of the epigenetic drivers of chordoma and their potential applications in prognosis and the development of new therapies.
脊索瘤是一种罕见的、生长缓慢的肉瘤，具有局部侵袭性，通常对传统的化疗和放疗有抵抗力。尽管发病率较低，但脊索瘤仍然是一个临床挑战，因为脊索瘤的治疗选择有限，而且对于涉及治疗耐药性的分子机制知之甚少。此外，目前还没有确定的预测或预后生物标志物来跟踪疾病进展或治疗。与其他癌症相比，脊索瘤组织的全基因组测序显示突变率较低。这引起了人们对表观遗传事件在脊索瘤发病机制中的作用的兴趣。在这篇综述中，我们讨论了目前对脊索瘤表观遗传驱动因素的认识及其在预后和新疗法开发中的潜在应用。
