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Multicenter Study
. 2016 Aug;174(3):417-24.
doi: 10.1111/bjh.14073. Epub 2016 Mar 28.

Trends in primary central nervous system lymphoma incidence and survival in the U.S

Affiliations
Multicenter Study

Trends in primary central nervous system lymphoma incidence and survival in the U.S

Meredith S Shiels et al. Br J Haematol. 2016 Aug.

Abstract

It is suspected that primary central nervous system lymphoma (PCNSL) rates are increasing among immunocompetent people. We estimated PCNSL trends in incidence and survival among immunocompetent persons by excluding cases among human immunodeficiency virus (HIV)-infected persons and transplant recipients. PCNSL data were derived from 10 Surveillance, Epidemiology and End Results (SEER) cancer registries (1992-2011). HIV-infected cases had reported HIV infection or death due to HIV. Transplant recipient cases were estimated from the Transplant Cancer Match Study. We estimated PCNSL trends overall and among immunocompetent individuals, and survival by HIV status. A total of 4158 PCNSLs were diagnosed (36% HIV-infected; 0·9% transplant recipients). HIV prevalence in PCNSL cases declined from 64·1% (1992-1996) to 12·7% (2007-2011), while the prevalence of transplant recipients remained low. General population PCNSL rates were strongly influenced by immunosuppressed cases, particularly in 20-39 year-old men. Among immunocompetent people, PCNSL rates in men and women aged 65+ years increased significantly (1·7% and 1·6%/year), but remained stable in other age groups. Five-year survival was poor, particularly among HIV-infected cases (9·0%). Among HIV-uninfected cases, 5-year survival increased from 19·1% (1992-1994) to 30·1% (2004-2006). In summary, PCNSL rates have increased among immunocompetent elderly adults, but not in younger individuals. Survival remains poor for both HIV-infected and HIV-uninfected PCNSL patients.

Keywords: Lymphoma; central nervous system; human immunodeficiency virus; incidence; survival; transplant.

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Figures

Figure 1
Figure 1
Age-standardized incidence rates of PCNSL overall and among immunocompetent individuals (i.e., excluding HIV-infected people and transplant recipients) in 10 SEER registries, 1992–2011. Solid lines indicate overall rates and dashed lines indicate immunompetent rates.
Figure 2
Figure 2
Age-standardized survival estimates for PCNSL cases diagnosed in 10 SEER registries during A) 1992–2011 and B) 1998–2011. Solid lines represent HIV-uninfected cases and dashed lines represent HIV-infected cases.
Figure 3
Figure 3
Age-standardized 5-year survival estimates for HIV-uninfected PCNSLs by 3-year categories of calendar year of diagnosis in 10 SEER registries during 1992–2005. Points represent estimates and dashed lines represent 95% confidence intervals.

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