Department of Medicine, Division of Hematology-Oncology, Northwestern University, Feinberg School of Medicine, 676 North St. Clair, suite 850, Chicago, IL 60611, USA.
Curr Treat Options Oncol. 2013 Jun;14(2):185-97. doi: 10.1007/s11864-013-0227-7.
Primary central nervous system lymphoma (PCNSL) comprises approximately 5 % of all primary brain tumors. During the past two decades the incidence of PCNSL has increased, and as a result clinical research to determine the optimal treatment for PCNSL patients also has increased. Diagnosis is based on histopathologic findings traditionally established by biopsy only. More recent data raise controversy and challenges this biopsy-only paradigm, showing a potential advantage for surgical resection with progression-free survival (PFS) and overall survival (OS). Using high-dose intravenous (IV) methotrexate-based chemotherapy alone or as part of a regimen can lead to disease cure. The role of whole brain radiotherapy (WBRT) remains controversial and more frequently is omitted to avoid potential delayed neurocognitive effects, especially in patients older than age 60 years. Newer data from Memorial Sloan Kettering Cancer Center (MSKCC) using five cycles of Rituximab, Methotrexate, Vincristine, and Procarbazine (R-MVP) followed by low-dose WBRT (2,340 cgy), and then two cycles of Ara-C had excellent disease control with low neuro-toxicity and is now the basis of an ongoing RTOG (Radiation Treatment Oncology Group) trial comparing early versus delayed WBRT. Other chemotherapeutics and novel treatments, such as autologous stem cell transplantation, are being studied for potential use in PCNSL. Unlike many other primary brain tumors seen in adults, PCNSL is potentially curable; therefore, balancing treatment decisions with long-term neurocognitive effects and toxicities is crucial.
原发性中枢神经系统淋巴瘤（PCNSL）约占所有原发性脑肿瘤的 5%。在过去的二十年中，PCNSL 的发病率有所增加，因此，为确定 PCNSL 患者的最佳治疗方法而进行的临床研究也有所增加。诊断基于传统上仅通过活检确定的组织病理学发现。最近的数据对这一仅活检的范例提出了争议和挑战，表明手术切除具有无进展生存期（PFS）和总生存期（OS）的潜在优势。单独使用大剂量静脉（IV）甲氨蝶呤为基础的化疗或作为方案的一部分，可以导致疾病治愈。全脑放疗（WBRT）的作用仍存在争议，更频繁地被省略以避免潜在的延迟神经认知影响，特别是在 60 岁以上的患者中。纪念斯隆凯特琳癌症中心（MSKCC）的最新数据使用五周期利妥昔单抗、甲氨蝶呤、长春新碱和丙卡巴肼（R-MVP），然后进行低剂量 WBRT（2,340 cgy），然后进行两个周期的阿糖胞苷，具有出色的疾病控制效果，且神经毒性低，目前正在进行一项 RTOG（放射治疗肿瘤学组）试验，比较早期与延迟 WBRT。其他化疗药物和新型治疗方法，如自体干细胞移植，正在研究用于 PCNSL 的潜在用途。与成年人中看到的许多其他原发性脑肿瘤不同，PCNSL 是可以治愈的；因此，平衡治疗决策与长期神经认知影响和毒性至关重要。
