Abstract：
Primary central nervous system lymphoma (PCNSL) is a type of central nervous system restricted non-Hodgkin lymphoma, whose histopathological diagnosis is majorly large B cell lymphoma. To provide specific, evidence-based recommendations for medical professionals and to promote more standardized, effective and safe treatment for patients with PCNSL, a panel of experts from the Chinese Neurosurgical Society of the Chinese Medical Association and the Society of Hematological Malignancies of the Chinese Anti-Cancer Association jointly developed an evidence-based consensus. After comprehensively searching literature and conducting systematic reviews, two rounds of Delphi were conducted to reach consensus on the recommendations as follows: The histopathological specimens of PCNSL patients should be obtained as safely and comprehensively as possible by multimodal tomography-guided biopsy or minimally invasive surgery. Corticosteroids should be withdrawn from, or not be administered to, patients with suspected PCNSL before biopsy if the patient\'s status permits. MRI (enhanced and DWI) should be performed for diagnosing and evaluating PCNSL patients where whole-body PET-CT be used at necessary time points. Mini-mental status examination can be used to assess cognitive function in the clinical management. Newly diagnosed PCNSL patients should be treated with combined high-dose methotrexate-based regimen and can be treated with a rituximab-inclusive regimen at induction therapy. Autologous stem cell transplantation can be used as a consolidation therapy. Refractory or relapsed PCNSL patients can be treated with ibrutinib with or without high-dose chemotherapy as re-induction therapy. Stereotactic radiosurgery can be used for PCNSL patients with a limited recurrent lesion who were refractory to chemotherapy and have previously received whole-brain radiotherapy. Patients with suspected primary vitreoretinal lymphoma (PVRL) should be diagnosed by vitreous biopsy. PVRL or PCNSL patients with concurrent VRL can be treated with combined systemic and local therapy.
摘要：
原发性中枢神经系统淋巴瘤(PCNSL)是一种中枢神经系统限制性非霍奇金淋巴瘤,其组织病理学诊断主要是大B细胞淋巴瘤。提供具体的,为医疗专业人员提供基于证据的建议，并促进更加标准化，为PCNSL患者提供有效和安全的治疗，中华医学会中国神经外科学会和中国抗癌协会血液恶性肿瘤学会的专家小组共同制定了基于证据的共识。在全面检索文献并进行系统综述后，我们进行了两轮Delphi研究,就以下建议达成共识:应通过多模态断层扫描引导活检或微创手术,尽可能安全全面地获取PCNSL患者的组织病理学标本.皮质类固醇应该退出，或者不被管理，如果患者状态允许，在活检前怀疑PCNSL的患者。应进行MRI（增强和DWI）以诊断和评估在必要时间点使用全身PET-CT的PCNSL患者。简易精神状态检查可用于临床管理中的认知功能评估。新诊断的PCNSL患者应采用基于甲氨蝶呤的联合大剂量方案治疗，并可在诱导治疗时采用利妥昔单抗包涵方案治疗。自体干细胞移植可作为一种巩固疗法。难治性或复发性PCNSL患者可以用依鲁替尼治疗，有或没有大剂量化疗作为再诱导疗法。立体定向放射外科可用于复发性病变有限的PCNSL患者，这些患者对化学疗法难以治疗，并且以前曾接受过全脑放射疗法。疑似原发性玻璃体视网膜淋巴瘤（PVRL）的患者应通过玻璃体活检进行诊断。并发VRL的PVRL或PCNSL患者可采用全身和局部联合治疗。
