Department of Neuro-Oncology, University and City of Health and Science Hospital of Turin, Turin, Italy.
Division of Neurology 2-Mazarin, Pitié Salpêtrière Hospital and Sorbonne University, Paris, France.
Curr Opin Neurol. 2020 Dec;33(6):707-715. doi: 10.1097/WCO.0000000000000866.
The management of low-grade (grade II) oligodendrogliomas is still controversial, due to their rarity and long-term survival. According to recent WHO 2016 Classification of central nervous system tumors oligodendrogliomas are defined by the coexistence of molecular alterations, such as isocitrate dehydrogenase (IDH)1/2 mutations and 1p/19q codeletion. These tumors have better outcome and higher response to chemotherapy compared with diffuse astrocytomas.
The association of radiotherapy and procarbazine, lomustine (CCNU), vincristine chemotherapy in low-grade oligodendrogliomas is definitely superior over radiotherapy alone, and yields median progression-free survival and overall survival values exceeding by far 10 years. Chemotherapy alone yields results that are inferior compared with radiotherapy + procarbazine, CCNU, vincristine but may better preserve cognitive functions from radiotherapy-induced damage. Chemosensitivity of oligodendrogliomas is related to a high percentage of O6-methylguanine-DNA methyltransferase methylation and low expression of DNA repair genes. Recurrent defects in mismatch repair pathways may induce hypermutation and secondary resistance to temozolomide, but not to nitrosoureas.
Reoperation at progression following initial chemotherapy is increasingly adopted, thus allowing a further delay of radiotherapy. In the future targeting IDH1/2 mutations following incomplete surgery may represent a new innovative option.
由于低级别（II 级）少突胶质细胞瘤的罕见性和长期生存，其治疗仍存在争议。根据最近的 2016 年世界卫生组织中枢神经系统肿瘤分类，少突胶质细胞瘤的定义是存在分子改变，如异柠檬酸脱氢酶（IDH）1/2 突变和 1p/19q 联合缺失。与弥漫性星形细胞瘤相比，这些肿瘤具有更好的预后和更高的化疗反应率。
低级别少突胶质细胞瘤中放疗联合洛莫司汀（CCNU）、长春新碱化疗明显优于单纯放疗，中位无进展生存期和总生存期均超过 10 年。与放疗+洛莫司汀、CCNU、长春新碱相比，单纯化疗的结果较差，但可能更好地保护认知功能免受放疗引起的损伤。少突胶质细胞瘤的化疗敏感性与 O6-甲基鸟嘌呤-DNA 甲基转移酶甲基化的高比例和 DNA 修复基因的低表达有关。错配修复途径的复发性缺陷可能导致对替莫唑胺的超突变和继发性耐药，但对亚硝脲类药物无耐药性。
初始化疗后进展时的再次手术越来越多地被采用，从而进一步延迟了放疗。未来，针对不完全手术后的 IDH1/2 突变可能代表一种新的创新选择。
