Fortin Ensign Shannon P, Gathers Diamone, Wiedmeier Julia Erin, Mrugala Maciej M
Department of Hematology and Oncology, Mayo Clinic Arizona, Phoenix, AZ, USA.
Department of Internal Medicine, University of New Mexico, Albuquerque, NM, USA.
Curr Treat Options Oncol. 2022 Jan;23(1):117-136. doi: 10.1007/s11864-021-00921-5. Epub 2022 Feb 19.
Primary central nervous system lymphomas (PCNSLs) are very rare neoplasms and continue to be challenging to treat. While high-dose methotrexate (HD-MTX)-based regimens are the currently accepted standard first-line therapy for newly diagnosed patients, the optimal induction therapies are still unknown. The role of consolidation therapies continues to evolve with a variety of chemotherapy regimens, including high-dose chemotherapy with stem cell rescue and reduced or deferred whole brain radiotherapy being used. Importantly, several recent advances have been made in the treatment of PCNSL. The incorporation of targeted therapy and immune therapy remain promising strategies. Several agents, successfully used in treatment of systemic lymphomas, have shown activity in PCNSL, frequently leading to durable responses in the relapsed/refractory patients. Many ongoing studies will likely lead to a better understanding of the roles of these treatments, especially as the first line and potentially also as maintenance. In addition, the use of molecular profiling to predict disease response to targeted agents and understand relapse patterns will become increasingly important. Clinical trials in PCNSL are critical yet frequently challenging to conduct given the rarity of the condition and lack of suitable subjects. Therefore, multi-institutional and international collaboration is of utmost importance to accelerate progress in understanding the biology and design better treatments for this disease. It is critical to consider patients of all demographics in the design and study of future treatment algorithms to have the largest impact on patient care and outcomes.
原发性中枢神经系统淋巴瘤（PCNSL）是非常罕见的肿瘤，治疗仍然具有挑战性。虽然基于大剂量甲氨蝶呤（HD-MTX）的方案是目前新诊断患者公认的标准一线治疗方法，但最佳诱导治疗方案仍不明确。巩固治疗的作用随着多种化疗方案的不断发展而变化，包括采用干细胞救援的大剂量化疗以及减少或推迟全脑放疗。重要的是，PCNSL的治疗最近取得了一些进展。靶向治疗和免疫治疗的纳入仍然是有前景的策略。几种成功用于治疗系统性淋巴瘤的药物在PCNSL中显示出活性，经常使复发/难治性患者产生持久反应。许多正在进行的研究可能会使人们更好地理解这些治疗的作用，特别是作为一线治疗以及可能作为维持治疗。此外，利用分子谱分析来预测疾病对靶向药物的反应并了解复发模式将变得越来越重要。鉴于PCNSL的罕见性和缺乏合适的受试者，PCNSL的临床试验至关重要但往往具有挑战性。因此，多机构和国际合作对于加速了解该疾病的生物学特性并设计更好的治疗方法至关重要。在未来治疗方案的设计和研究中考虑所有人口统计学特征的患者对于对患者护理和结果产生最大影响至关重要。
