Abstract
ObjectiveThis study was conducted in order to study the clinical characteristics, prognostic factors, and treatment outcomes in patients with primary central nervous system lymphoma (PCNSL).Materials and MethodsThe data of a total of 5,166 PCNSL patients diagnosed between 2000 and 2018 from the Surveillance, Epidemiology, and End Results (SEER) database were obtained.ResultsThe mean age was 63.1 ± 14.9 years, with a male to female ratio of 1.1:1.0. The most common histologic subtype was diffuse large B-cell lymphoma (DLBCL) (84.6%). The 1-, 3-, and 5-year overall survival (OS) rates were 50.1%, 36.0%, and 27.2%, respectively, and the corresponding disease-specific survival (DSS) rates were 54.4%, 41.3%, and 33.5%, respectively. Multivariate analysis with Cox regression showed that race, sex, age, marital status, surgical resection, and chemotherapy were independent prognostic factors for OS and DSS, but radiotherapy was only for OS. Nomograms specially for DLBCL were established to predict the possibility of OS and DSS. The concordance index (C-index) values of OS and DSS were 0.704 (95% CI 0.687–0.721) and 0.698 (95% CI 0.679–0.717), suggesting the high discrimination ability of the nomograms.ConclusionSurgical resection and/or chemotherapy was favorably associated with better OS and DSS. However, radiotherapy was not beneficial for OS and DSS in the long term. A new predictive nomogram and a web-based survival rate calculator we developed showed favorable applicability and accuracy to predict the long-term OS for DLBCL patients specifically.
Highlights
Primary central nervous system lymphoma (PCNSL) is an uncommon and highly invasive tumor that involves the leptomeninges, brain, eyes, or spinal cord without evidence of systemic disease [1]
5,166 PCNSL patients were included in the study
We explored the effects of combination therapy and found that surgical resection combined with chemotherapy was associated with better overall survival (OS) and DSS (Figures 4A, B), but radiotherapy combined with chemotherapy led to worse OS and DSS in the long term (Figures 4C, D)
Summary
Primary central nervous system lymphoma (PCNSL) is an uncommon and highly invasive tumor that involves the leptomeninges, brain, eyes, or spinal cord without evidence of systemic disease [1]. PCNSL accounts for 1%–2% of nonHodgkin lymphoma (NHL) and most of the cases (over 90%) are diffuse large B-cell lymphoma (DLBCL) [2, 3]. Immunocompromised individuals, such as HIV-infected or immunosuppressive patients, are deemed to have a higher risk of developing PCNSL [4, 5]. High-dose methotrexate (HD-MTX) systemic chemotherapy is deemed as the standard first-line treatment; few patients can achieve long-term survival: the median progression‐free survival (PFS) and OS were only 24.0 and 36.9 months, respectively [6, 7], and the 5-year OS in the period 1992–1994 was increased only from 19.1% to 30.1% in the period 2004–2006 [4]. Even with an impressive clinical response [8,9,10,11], more randomized clinical trials are still needed to verify and identify the optimal therapy for PCNSL patients
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