APHP, Department of Neurology, Groupe Hospitalier Pitié-Salpêtrière; Sorbonne Université; IHU; ICM. Paris, France.
Institute of Neuropathology, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.
Neuro Oncol. 2023 Jan 5;25(1):37-53. doi: 10.1093/neuonc/noac196.
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.
原发性中枢神经系统淋巴瘤（PCNSL）的治疗是神经肿瘤学中最具争议的话题之一，这是由于疾病的复杂性和可用的对照研究数量有限所致。2021 年，鉴于最近的进展和改变实践的随机试验的发表，欧洲神经肿瘤学会（EANO）成立了一个多学科工作组，以更新之前发表的针对免疫功能正常的 PCNSL 成年患者的循证指南，并增加了免疫抑制患者的章节。该指南提供了 PCNSL 治疗的共识性考虑因素和建议，包括眼内表现和老年患者的具体管理。与之前的指南相比，主要变化包括：在一线治疗中强化巩固治疗与 ASCT 的证据，前瞻性评估年轻和老年患者的化疗联合方案，即使数据仍不确定，也明确利妥昔单抗的作用，明确新药物的作用，纳入免疫抑制患者和原发性眼内淋巴瘤。该指南应有助于临床医生在日常实践和决策中，并为该领域的未来研究提供基础。
