脊索瘤分子调控机制的研究进展

Research Advances in Molecular Regulatory Mechanism of Chordoma

脊索瘤是起源于胚胎残余脊索组织的原发性恶性肿瘤。由于脊索瘤局部侵袭性高及化疗耐受性强,其复发率较高,预后较差。虽然相关研究发现原癌基因Brachyury等多个分子参与脊索瘤发生发展,然而其病因仍不明确。脊索瘤的治疗仍然是困扰临床医生的难题。近年来,新的分子标志物iASPP、SMARCB1及信号通路PI3K/AKT等被证实与脊索瘤的发生相关。确定关键分子及信号通路,一方面有助于深入了解脊索瘤的病因及发病机制,另一方面也为研发新的靶向药物以改善脊索瘤治疗现状奠定基础。本文从目前研究较多的关键分子、信号通路及表观遗传学等方面对脊索瘤分子调控机制的研究进展进行综述。

Chordoma is a rare primary malignant bone tumor derived from notochord remnants.Due to its strong local invasiveness and chemotherapy resistance,chordoma has a high local recurrence rate and poor prognosis.Although previous studies identified many molecules including brachyury as the oncogenes in chordoma,the pathogenesis of chordoma remains to be fully elucidated.The treatment of chordoma is still a challenge for the surgeons.In recent year,multiple novel biomarkers such as iASPP,SMARCB1 and signaling pathways like PI3K/AKT have been reported to be related with the pathogenesis of chordoma.Identifying crucial targets and signaling pathways is helpful to understand the pathogenesis of chordoma further,and lays a solid foundation to develop new drugs to improve the therapeutic effects of chordoma on the other hand.In this paper,we aim to review the recent advances in molecular regulation mechanism of chordoma.