{"title":"Central Nervous System Lymphoma.","authors":"Lauren Schaff","doi":"10.1212/CON.0000000000001356","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>This article reviews the clinical presentation, diagnostic workup, staging, and treatment of primary central nervous system (CNS) lymphoma and common manifestations of secondary CNS lymphoma.</p><p><strong>Latest developments: </strong>Lymphoma can arise in the CNS de novo (primary CNS lymphoma) or as the result of systemic disease (secondary CNS lymphoma). Symptoms may include focal neurologic deficits related to the disease site, cognitive decline, and symptoms of increased intracranial pressure. Standard treatment may differ based on lymphoma subtype and location. A majority of CNS lymphoma is diffuse large B-cell subtype and exhibits aggressive behavior. First-line treatment is generally methotrexate-based polychemotherapy. Response rates to treatment are high, approximately 80% to 90% for primary CNS lymphoma, but relapse is common. Consolidation approaches including myeloablative chemotherapy followed by autologous stem cell rescue, nonmyeloablative chemotherapy, radiation, and medical maintenance regimens reduce rates of relapse. The recent development of targeted agents such as Bruton tyrosine kinase inhibitors and immunomodulatory strategies have shown promise in the treatment of CNS lymphoma. Immunotherapy in the form of checkpoint inhibitors and chimeric antigen receptor T cells is being studied. More indolent forms of lymphoma may be treated with radiation or targeted therapy.</p><p><strong>Essential points: </strong>CNS lymphoma is an uncommon but clinically meaningful manifestation of extranodal lymphoma. The diagnosis requires a high level of suspicion for rapid initiation of potentially curative treatment.</p>","PeriodicalId":52475,"journal":{"name":"CONTINUUM Lifelong Learning in Neurology","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"CONTINUUM Lifelong Learning in Neurology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1212/CON.0000000000001356","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
Abstract
Objective: This article reviews the clinical presentation, diagnostic workup, staging, and treatment of primary central nervous system (CNS) lymphoma and common manifestations of secondary CNS lymphoma.
Latest developments: Lymphoma can arise in the CNS de novo (primary CNS lymphoma) or as the result of systemic disease (secondary CNS lymphoma). Symptoms may include focal neurologic deficits related to the disease site, cognitive decline, and symptoms of increased intracranial pressure. Standard treatment may differ based on lymphoma subtype and location. A majority of CNS lymphoma is diffuse large B-cell subtype and exhibits aggressive behavior. First-line treatment is generally methotrexate-based polychemotherapy. Response rates to treatment are high, approximately 80% to 90% for primary CNS lymphoma, but relapse is common. Consolidation approaches including myeloablative chemotherapy followed by autologous stem cell rescue, nonmyeloablative chemotherapy, radiation, and medical maintenance regimens reduce rates of relapse. The recent development of targeted agents such as Bruton tyrosine kinase inhibitors and immunomodulatory strategies have shown promise in the treatment of CNS lymphoma. Immunotherapy in the form of checkpoint inhibitors and chimeric antigen receptor T cells is being studied. More indolent forms of lymphoma may be treated with radiation or targeted therapy.
Essential points: CNS lymphoma is an uncommon but clinically meaningful manifestation of extranodal lymphoma. The diagnosis requires a high level of suspicion for rapid initiation of potentially curative treatment.
目的:本文回顾了原发性中枢神经系统(CNS)淋巴瘤的临床表现、诊断、分期和治疗以及继发性中枢神经系统淋巴瘤的常见表现:本文回顾了原发性中枢神经系统(CNS)淋巴瘤的临床表现、诊断、分期和治疗,以及继发性中枢神经系统淋巴瘤的常见表现:淋巴瘤可在中枢神经系统新发(原发性中枢神经系统淋巴瘤)或因全身性疾病而发生(继发性中枢神经系统淋巴瘤)。症状可能包括与发病部位有关的局灶性神经功能缺损、认知能力下降和颅内压增高症状。标准治疗可能因淋巴瘤亚型和发病部位而异。大多数中枢神经系统淋巴瘤属于弥漫大 B 细胞亚型,具有侵袭性。一线治疗一般采用以甲氨蝶呤为基础的多化学疗法。治疗反应率很高,原发性中枢神经系统淋巴瘤的反应率约为80%至90%,但复发很常见。巩固治疗方法包括髓脱落化疗后的自体干细胞救治、非髓脱落化疗、放疗和药物维持治疗,这些方法可降低复发率。最近开发的布鲁顿酪氨酸激酶抑制剂等靶向药物和免疫调节策略在中枢神经系统淋巴瘤的治疗中大有可为。目前正在研究检查点抑制剂和嵌合抗原受体 T 细胞等形式的免疫疗法。较不活跃的淋巴瘤可采用放射治疗或靶向治疗:要点:中枢神经系统淋巴瘤是结节外淋巴瘤的一种不常见但有临床意义的表现。诊断时需要高度怀疑,以便迅速开展可能治愈的治疗。
期刊介绍:
Continue your professional development on your own schedule with Continuum: Lifelong Learning in Neurology®, the American Academy of Neurology" self-study continuing medical education publication. Six times a year you"ll learn from neurology"s experts in a convenient format for home or office. Each issue includes diagnostic and treatment outlines, clinical case studies, a topic-relevant ethics case, detailed patient management problem, and a multiple-choice self-assessment examination.