{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:46:13Z","timestamp":1790837173085,"version":"4.1.0"},"reference-count":89,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2018,1,1]],"date-time":"2018-01-01T00:00:00Z","timestamp":1514764800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Neurol Disord"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:p> Primary central nervous system lymphoma (PCNSL) is a rare and aggressive extranodal non-Hodgkin lymphoma (NHL), confined to the brain, eyes, spinal cord or leptomeninges without systemic involvement. Overall prognosis, diagnosis and management of PCNSL differ from other types of NHL. Prompt diagnosis and initiation of treatment are vital to improving clinical outcomes. PCNSL is responsive to radiation therapy, however whole-brain radiotherapy (WBRT) inadequately controls the disease when used alone and its delayed neurotoxicity causes neurocognitive impairment, especially in elderly patients. High-dose methotrexate (HD-MTX)-based induction chemotherapy with or without autologous stem cell transplantation (ASCT) or reduced-dose WBRT leads to durable disease control and less neurotoxicity. The optimal treatment has yet to be defined, however HD-MTX-based induction chemotherapy is considered standard for newly diagnosed PCNSL. Ongoing randomized trials address the role of rituximab, and of consolidative treatment using ASCT or reduced-dose WBRT. Despite high tumor response rates to initial treatment, many patients have relapsing disease with very poor prognosis. The optimal treatment for refractory or relapsed PCNSL is poorly defined. The choice of salvage treatment depends on age, previous treatment and response, performance status and comorbidities at the time of relapse. Novel therapeutics targeting underlying tumor biology include small molecule inhibitors of B-cell receptor, cereblon, and mammalian target of rapamycin signaling, and immunotherapy programmed cell death 1 receptor inhibitors and chimeric antigen receptor T cells. <\/jats:p>","DOI":"10.1177\/1756286418793562","type":"journal-article","created":{"date-parts":[[2018,10,5]],"date-time":"2018-10-05T13:17:05Z","timestamp":1538745425000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":64,"title":["Primary central nervous system lymphoma"],"prefix":"10.1177","volume":"11","author":[{"given":"Sarah","family":"L\u00f6w","sequence":"first","affiliation":[{"name":"Neurology Clinic and National Center for Tumor Diseases, University Hospital Heidelberg, Heidelberg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Catherine H.","family":"Han","sequence":"additional","affiliation":[{"name":"Auckland Cancer Society Research Centre, School of Medical Sciences, Faculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7568-6607","authenticated-orcid":false,"given":"Tracy T.","family":"Batchelor","sequence":"additional","affiliation":[{"name":"Stephen E. and Catherine Pappas Center for Neuro-Oncology, Yawkey 9E, 55 Fruit Street, Boston, MA 02114, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2018,10,5]]},"reference":[{"key":"bibr1-1756286418793562","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nov189."},{"key":"bibr2-1756286418793562","volume-title":"Lymphoma and leukemia of the nervous system","author":"Batchelor TT","year":"2013"},{"key":"bibr3-1756286418793562","unstructured":"Swerdlow SH, Campo E, Harris NL, et al. 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