Rare central nervous system lymphomas.

Central nervous system (CNS) lymphomas are rare malignancies characterised by lymphoid infiltration into the brain, spinal cord, cranial nerves, meninges and/or eyes in the presence or absence of previous or concurrent systemic disease. Most CNS lymphomas are of the diffuse large B-cell lymphoma (DLBCL) subtype for which treatment strategies, particularly the use of high-dose methotrexate-based protocols and consolidation with autologous stem cell transplantation, are well established. Other histopathological subtypes of CNS lymphoma are comparatively less common with published data on these rare lymphomas dominated by smaller case series and retrospective reports. Consequently, there exists little clinical consensus on the optimal methods to diagnose and manage these clinically and biologically heterogeneous CNS lymphomas. In this review article, we focus on rarer CNS lymphomas, summarising the available clinical data on incidence, context, diagnostic features, reported management strategies, and clinical outcomes.

Improved survival outcomes despite older age at diagnosis: an era-by-era analysis of patients with primary central nervous system lymphoma treated at a single referral centre in the United Kingdom.

Observational studies with long-term follow-up of patients with primary central nervous system lymphoma (PCNSL) are scarce. Patient data over a period of four decades were retrospectively analysed from databases at Nottingham University Hospitals Trust, UK. The cohort was delineated by two distinct therapeutic eras; the first from 01/01/1982 to 31/12/2010 (n = 147) and the second 01/01/2011 to 31/07/2020 (n = 125). The median age at diagnosis was significantly older in the second era compared to the first (69 and 65 years respectively, P = 0·003). The 3-, 6- and 12-month overall survival (OS) rates in the second era were significantly higher compared to the first, at 85%, 77%, 62% versus 56%, 49%, 38% respectively (log-rank test P < 0·0001). On multivariate analysis, high-dose methotrexate (HD-MTX)-based induction protocols employed in the second era were associated with improved OS compared to those used in the first [hazard ratio (HR) 0·40, 95% confidence interval (CI) 0·28-0·57]. Within the second era, superior OS rates were seen with the use of intensive HD-MTX protocols (including consolidation with high-dose chemotherapy and autologous stem cell transplantation) compared to non-intensive HD-MTX schedules (HR 0·47, 95% CI 0·22-0·99). Initiating chemotherapy within 14 days of biopsy and use of rituximab were independently associated with improved OS and progression-free survival during the second era. These data suggest that prompt treatment initiation and use of intensive HD-MTX- and rituximab-based protocols have resulted in improved survival outcomes for patients.

Adherence in leading medical journals to the CONSORT 2010 statement for reporting of binary outcomes in randomised controlled trials: cross-sectional analysis.

Clinicians and lay people tend to overestimate the effectiveness of a treatment when only the relative effect is presented, particularly if the relative effect is large, but the absolute effect is small. In recognition of this problem, item 17b of The Consolidated Standards of Reporting Trials (CONSORT) 2010 statement stipulates authors present both absolute and relative effects for binary outcomes in randomised controlled trials (RCTs). Adherence to item 17b and the effect of differing levels of CONSORT endorsement by journals on adherence is not well known. We assessed the extent to which item 17b is adhered to in 258 RCTs published in five leading medical journals (Annals of Internal Medicine, BMJ, JAMA, The Lancet and The New England Journal of Medicine) between January and December 2019 that all endorsed the CONSORT statement to varying degrees. Only 53 of 258 (20.5%; 95% CI 15.8% to 26.0%) included studies adhered fully to item 17b. Proportional adherence was higher in journals that endorsed the statement more strictly (BMJ and JAMA, 47.4% [34.0% to 61.0%]) compared with journals less strict in their endorsement (NEJM and Ann Intern Med, 12.2% [7.0% to 19.3%]; The Lancet, 14.1% [7.3% to 23.8%]). Journals that only recommend author adherence to CONSORT had a greater proportion of studies reporting only relative effects in the main results section (62.6%) and abstract (64.2%) compared with journals that require authors to submit a completed checklist (24.6% and 29.8%, respectively). The majority of RCTs (79.5%) with binary primary outcomes published in five leading medical journals during 2019 do not report both absolute and relative effect estimates as per item 17b of the CONSORT guideline despite its universal endorsement. Differences in adherence were observed between journals that endorsed the CONSORT statement to differing extents.