Harder Anja
Institute of Pathology, Martin Luther University Halle-Wittenberg, Magdeburger Str. 14, 06120, Halle (Saale), Germany.
Institute of Neuropathology, University Hospital Münster, Münster, Germany.
Biomark Res. 2021 Apr 16;9(1):26. doi: 10.1186/s40364-021-00281-0.
MAP/ERK kinase 1 and 2 (MEK 1/2) inhibitors (MEKi) are investigated in several trials to treat lesions that arise from pathogenic variants of the Neurofibromatosis type 1 and type 2 genes (NF1, NF2). These trials showed that MEKi are capable to shrink volume of low grade gliomas and plexiform neurofibromas in NF1. Targeting other lesions being associated with a high morbidity in NF1 seems to be promising. Due to involvement of multiple pathways in NF2 associated lesions as well as in malignant tumors, MEKi are also used in combination therapies. This review outlines the current state of MEKi application in neurofibromatosis and associated benign and malignant lesions.
丝裂原活化蛋白激酶/细胞外信号调节激酶1和2（MEK 1/2）抑制剂（MEKi）正在多项试验中进行研究，以治疗由1型和2型神经纤维瘤病基因（NF1、NF2）的致病变体引起的病变。这些试验表明，MEKi能够缩小NF1中低级别胶质瘤和丛状神经纤维瘤的体积。针对NF1中其他具有高发病率的病变似乎很有前景。由于NF2相关病变以及恶性肿瘤中涉及多种途径，MEKi也被用于联合治疗。本综述概述了MEKi在神经纤维瘤病及相关良性和恶性病变中的应用现状。
