Department of Hematology, Institute of Medicine, Huashan Hospital, Fudan University, Shanghai, 200040, China.
Department of Hematology, Beijing Tiantan Hospital, Capital Medical University, Beijing, 100070, China.
J Hematol Oncol. 2022 Sep 29;15(1):136. doi: 10.1186/s13045-022-01356-7.
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 Corticosteroids should be withdrawn from, or not be administered to, patients with suspected PCNSL before biopsy if the patient's status permits.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.
原发性中枢神经系统淋巴瘤（PCNSL）是一种中枢神经系统局限的非霍奇金淋巴瘤，其组织病理学诊断主要为大 B 细胞淋巴瘤。为了为医务人员提供具体的、基于证据的建议，并促进 PCNSL 患者更标准化、更有效和更安全的治疗，中华医学会神经外科学分会和中国抗癌协会血液肿瘤专业委员会的专家组共同制定了一个基于证据的共识。通过全面检索文献并进行系统评价，进行了两轮 Delphi 调查，就以下建议达成共识：PCNSL 患者的组织病理学标本应尽可能通过多模态断层扫描引导活检或微创手术安全且全面地获取。如果患者情况允许，在活检前，疑似 PCNSL 的患者应停止或不给予皮质类固醇。应在必要的时间点进行全身 PET-CT 检查以诊断和评估 PCNSL 患者，并进行 MRI（增强和 DWI）检查。在临床管理中，可以使用简易精神状态检查来评估认知功能。新诊断的 PCNSL 患者应接受包含高剂量甲氨蝶呤的联合方案治疗，并可在诱导治疗中接受包含利妥昔单抗的方案治疗。自体造血干细胞移植可作为巩固治疗。复发或难治性 PCNSL 患者可以用伊布替尼联合或不联合大剂量化疗作为再诱导治疗。对于对化疗耐药且既往接受过全脑放疗的有限复发性病变的 PCNSL 患者，可以使用立体定向放射外科治疗。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。对于并发 VRL 的 PVRL 或 PCNSL 患者，可以采用系统和局部联合治疗。
