Park Meerim
Department of Pediatrics, Center for Pediatric Cancer, National Cancer Center, Goyang, Korea.
Brain Tumor Res Treat. 2022 Apr;10(2):101-107. doi: 10.14791/btrt.2022.0006.
Optic pathway gliomas (OPGs) are insidious, debilitating low-grade tumors. They can affect the optic nerve, optic chiasm, and optic tracts and can be sporadic or associated with neurofibromatosis type 1 (NF1). The location of OPGs within the optic pathway typically precludes complete resection or optimal radiation dose. Treatment is unnecessary for sporadic and NF1-related OPGs that do not cause visual impairments. Chemotherapy is the mainstay of treatment for patients with progressive disease. However, outcomes following standard treatments have been mixed, and standardized outcome measurements are lacking. In recent years, newer molecularly targeted therapies such as anti-vascular endothelial growth factor (VEGF) monoclonal antibody, mitogen-activated protein kinase (MAPK) inhibitor, and mammalian target of rapamycin (mTOR) inhibitor, represent a promising treatment modality.
视路胶质瘤(OPG)是隐匿性、使人衰弱的低度肿瘤。它们可影响视神经、视交叉和视束,可为散发性或与1型神经纤维瘤病(NF1)相关。OPG在视路中的位置通常使完全切除或给予最佳辐射剂量难以实现。对于不引起视力损害的散发性和NF1相关OPG,无需治疗。化疗是疾病进展患者的主要治疗方法。然而,标准治疗后的结果不一,且缺乏标准化的疗效评估指标。近年来,抗血管内皮生长因子(VEGF)单克隆抗体、丝裂原活化蛋白激酶(MAPK)抑制剂和雷帕霉素靶蛋白(mTOR)抑制剂等新型分子靶向疗法是一种有前景的治疗方式。