Patrick Lauren B, Mohile Nimish A
Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 356, Rochester, NY, 14642, USA.
Department of Neurology, University of Rochester Medical Center, 601 Elmwood Avenue, Box 704, Rochester, NY, 14642, USA.
Curr Oncol Rep. 2015 Dec;17(12):60. doi: 10.1007/s11912-015-0483-8.
Primary central nervous system lymphoma (PCNSL) is a rare form of non-Hodgkin lymphoma that is limited to the CNS. Although novel imaging techniques aid in discriminating lymphoma from other brain tumors, definitive diagnosis requires brain biopsy, vitreoretinal biopsy, or cerebrospinal fluid analysis. Survival rates in clinical studies have improved over the past 20 years due to the addition of high-dose methotrexate-based chemotherapy regimens to whole-brain radiotherapy. Long-term survival, however, is complicated by clinically devastating delayed neurotoxicity. Newer regimens are attempting to reduce or eliminate radiotherapy from first-line treatment with chemotherapy dose intensification. Significant advances have also been made in the fields of pathobiology and treatment, with more targeted treatments on the horizon. The rarity of the disease makes conducting of prospective clinical trials challenging, requiring collaborative efforts between institutions. This review highlights recent advances in the biology, detection, and treatment of PCNSL in immunocompetent patients.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见的非霍奇金淋巴瘤，局限于中枢神经系统。尽管新型成像技术有助于鉴别淋巴瘤与其他脑肿瘤，但明确诊断仍需要进行脑活检、玻璃体视网膜活检或脑脊液分析。在过去20年中，由于在全脑放疗中加入了以大剂量甲氨蝶呤为基础的化疗方案，临床研究中的生存率有所提高。然而，长期生存因临床上具有破坏性的迟发性神经毒性而变得复杂。新的治疗方案正试图通过强化化疗剂量来减少或避免一线治疗中的放疗。在病理生物学和治疗领域也取得了重大进展，更具针对性的治疗即将出现。该疾病的罕见性使得开展前瞻性临床试验具有挑战性，需要各机构之间的合作。本综述重点介绍了免疫功能正常患者PCNSL在生物学、检测和治疗方面的最新进展。
