间变性淋巴瘤激酶与神经母细胞瘤关系的研究进展

Correlation between Anaplastic Lymphoma Kinase and Neuroblastoma

神经母细胞瘤(neuoblastonma,NB)是小儿最常见的实体肿瘤之一,临床表现变异较大,高危NB进展迅速,即使行高强度清髓化疗,肿瘤复发仍然常见,死亡率较高为发现有效NB治疗药物和靶点,必须充分研究认识NB发病机制中的关键分子。间变性淋巴瘤激酶(ALK)是一种受体型酪氨酸激酶,其异常存在于多种肿瘤中,与肿瘤发生发展密切相关,如间变性大细胞淋巴瘤、横纹肌肉瘤、炎症性肌纤维母细胞瘤、NB。ALK的异常形式主要包括基因融合、基因突变、基因扩增及蛋白表达增加随着酪氨酸激酶抑制剂在临床抗肿瘤治疗中的应用以及新的特异性小分子ALK抑制剂的研发,ALK异常与NB发生发展关系及针对ALK异常的NB靶向治疗获得了较多关注和研究。

Neuroblastoma （ NB ）, one of the most common solid tumors in children, has widely varied clinical manifestations. High-risk NB progresses rapidly; even with intensive myeloablative chemotherapy, re/apse is still common and almost all are fatal. To discover effective drugs and improve the prognosis of NB, the critical molecules in the pathogenesis of NB need to be examined. Anaplastic lymphoma kinase （ ALK ） is a member of the insulin receptor superfamily of receptor tyrosine kinases. ALK abnormalities exist in a variety of tumors, such as anaplastic large cell lymphoma, rhabdomyosarcoma, inflammatory myofibroblastic tumor, and NB. The abnormal forms of ALK include gene fusion, gene amplification, protein overexpression, and gene mutation. These abnormalities play important roles in the development of these tumors. Tyrosine kinase inhibitors as anti-cancer therapy for clinical applications and new specific small-molecule inhibitors of ALK have been developed. Consequently, the relationships of ALK aberrations with NB development and targeted ALK treatments for NB have received increased attention. This article mainly reviews the studies on the relationship between ALK abnormalities and NB development.