脊索瘤信号通路与分子靶向治疗

脊索瘤是一种少见的骨组织恶性肿瘤，来源于残留胚胎脊索组织，占骨原发恶性肿瘤的1%~4%。主要发生于颅底、脊柱及骶尾部的中轴骨组织。肿瘤发生部位特殊，故手术完整切除困难，而传统放疗及化疗的效果亦欠佳，因此脊索瘤常常侵犯周围组织，并多次复发。近年来关于脊索瘤的分子生物学方面研究日益深入，受体酪氨酸激酶及其下游信号通路Akt/mTOR、Ras/MEK/ERK，以及其他信号通路，如STAT3、NF-κB等在脊索瘤中相继被发现，相关分子靶向药物的研究也进入Ⅰ期和Ⅱ期临床试验阶段。本文就目前研究较多的几条脊索瘤信号通路及分子靶向治疗进展作一综述。

Chordoma Signaling Pathways and Molecular Targeted Therapy

Chordoma is a rare primary bone malignancy, accounting for 1%-4% of all bone primary malignant tumors, which is believed to arise from the remnants of notochord tissues. These tumors typically occur in the axial skeleton, most commonly in the sacrum, skull base and spine. Because of the special location, complete surgical resection and delivery of definitive radiation are not feasible, while conventional chemotherapeutic agents are also not effective. These tumors tend to be locally invasive with a high rate of recurrence. Recent studies have begun to characterize the molecular biology of chordoma. Receptor tyrosine kinase, Akt/mTOR, Ras/MEK/ERK, STAT3 and NF-κB signaling pathways in chordoma have been found. Several molecular targeted drugs for potential clinical therapies have been determined. This article will describe some main signaling pathways in chordoma and the progress of relevant molecular targeted therapy.