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近年来儿童恶性肿瘤的发病率有逐年增高的趋势,高危患儿预后差,且手术及放化疗等主要治疗手段的疗效已经达到平台期。靶向治疗以其可靠的疗效和轻微的毒副反应成为目前最受关注和最有前景的治疗手段之一。间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)是一种受体型酪氨酸激酶,近年的研究成果表明其异常与多种儿童肿瘤的发生发展密切相关。ALK的异常形式主要包括基因融合、基因突变、基因扩增和蛋白表达增加。随着ALK抑制剂在临床抗肿瘤治疗中的应用,针对ALK异常的靶向治疗获得越来越多的关注。本研究主要针对ALK在儿童恶性肿瘤中的异常以及与其发生发展关系的研究进展进行综述。
In recent years, the incidence of childhood malignancies have increased year by year trend, high-risk children with poor prognosis, and the efficacy of major surgical and radiotherapy and chemotherapy treatment has reached a platform period. Targeted therapy with its reliable efficacy and mild side effects become the most attention and the most promising treatment. Anaplastic lymphoma kinase (ALK) is a kind of receptor tyrosine kinase. Recent researches have shown that its abnormality is closely related to the occurrence and development of many kinds of childhood tumors. Abnormal forms of ALK mainly include gene fusion, gene mutation, gene amplification and increased protein expression. With the application of ALK inhibitors in clinical anti-tumor therapy, more and more attention has been paid to targeted therapy of ALK abnormalities. This study mainly reviews the research progress of the abnormalities of ALK in children with malignant tumors and the relationship with the occurrence and development of ALK.