由于疾病的复杂性和可用的对照研究数量有限，原发性中枢神经系统（PCNSL）的管理是神经肿瘤学中最有争议的主题之一。2021年，鉴于最新进展和改变实践的随机试验的出版，欧洲神经肿瘤协会(EANO)成立了一个多学科工作组,以更新先前发表的针对有免疫能力的成人PCNSL患者的循证指南,并增加了关于免疫抑制患者的章节.该指南为PCNSL的治疗提供了共识考虑和建议，包括老年人眼内表现和具体管理。与以前的指南相比，主要的变化包括加强了ASCT在一线治疗中巩固的证据，前瞻性评估年轻和老年患者的化疗组合，澄清利妥昔单抗的作用，即使数据仍不确定，新代理人的角色，以及合并免疫抑制患者和原发性眼部淋巴瘤。该指南应帮助临床医生进行日常实践和决策，并作为该领域未来研究的基础。
The management of primary central nervous system (PCNSL) is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the limited number of controlled studies available. In 2021, given recent advances and the publication of practice-changing randomized trials, the European Association of Neuro-Oncology (EANO) created a multidisciplinary task force to update the previously published evidence-based
guidelines for immunocompetent adult patients with PCNSL and added a section on immunosuppressed patients. The
guideline provides
consensus considerations and recommendations for the treatment of PCNSL, including intraocular manifestations and specific management of the elderly. The main changes from the previous
guideline include strengthened evidence for the consolidation with ASCT in first-line treatment, prospectively assessed chemotherapy combinations for both young and elderly patients, clarification of the role of rituximab even though the data remain inconclusive, of the role of new agents, and the incorporation of immunosuppressed patients and primary ocular lymphoma. The
guideline should aid the clinicians in everyday practice and decision making and serve as a basis for future research in the field.
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