PURPOSE This study aimed to provide an updated overview of primary central lymphoma (PCL) using a large cohort of 33 years. That being said, we attempted to examine the patient demographics, management plans and their outcome, causes of death and the time trends in overall incidence and mortality rates of these patients. METHODS We conducted a retrospective cohort study using the Surveillance, Epidemiology, and End Results (SEER) database between 1983 and 2016. We calculated the frequencies and the average annual age-adjusted rate (AAR) of PCL patients. Uni- and multivariable accelerated failure time regression were used to identify possible prognostic factors affecting the patients' survival. Furthermore, detailed causes of death were extracted and joint point regression analysis was done to examine incidence and mortality trends. RESULTS We identified 2925 PCL cases. The AAR was 0.148 per 100,000. An increase in age was significantly associated with shorter survival (HR: 1.01, 95%CI = 1.01-1.01, P < .001), while a recent year of diagnosis after 1993-2002 and 2002 was associated with improved survival (HR: 0.76, CI = 0.65-0.89, P = .001), and (HR: 0.48, CI = 0.41-0.56, P < .001), respectively. Overall, the trend of mortality rates in PCL patients has declined over the past years (-1.38% per year). CONCLUSION Our results support the previous evidence by showing an increase in patients' survival over time. While most PCL-related deaths occur within the first year, subsequent slow progression was observed after the first few years of survival. More attention should be given to the other possible non-PCL causes of death, especially beyond ten years of survival.

中文翻译：

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美国原发性脑淋巴瘤的特征、发病率、存活率和死亡原因

目的 本研究旨在使用 33 年的大型队列提供原发性中央淋巴瘤 (PCL) 的最新概述。话虽如此，我们试图检查患者的人口统计学、管理计划及其结果、死亡原因以及这些患者的总体发病率和死亡率的时间趋势。方法 我们使用 1983 年至 2016 年间的监测、流行病学和最终结果 (SEER) 数据库进行了一项回顾性队列研究。我们计算了 PCL 患者的频率和年均年龄调整率 (AAR)。使用单变量和多变量加速失败时间回归来确定可能影响患者生存的预后因素。此外，还提取了详细的死因，并进行了联合点回归分析以检查发病率和死亡率趋势。结果 我们确定了 2925 个 PCL 病例。AAR 为每 100,000 人 0.148。年龄增加与生存期缩短显着相关（HR：1.01，95%CI = 1.01-1.01，P < .001），而 1993-2002 和 2002 年之后的最近一年诊断与生存期改善相关（HR：0.76 , CI = 0.65-0.89, P = .001) 和 (HR: 0.48, CI = 0.41-0.56, P < .001)。总体而言，PCL 患者的死亡率在过去几年中呈下降趋势（每年 -1.38%）。结论 我们的结果通过显示患者存活率随着时间的推移而增加来支持先前的证据。虽然大多数 PCL 相关死亡发生在第一年内，但在存活的最初几年后观察到随后的缓慢进展。应更多关注其他可能的非 PCL 死亡原因，尤其是超过十年的生存期。