Primary Central Nervous System Lymphoma Increasing Incidence and Poor Survival: A Finnish Population-based Analysis

Abstract PurposeWe report here the first population-based incidence rates and prognosis of primary central nervous system lymphoma (PCNSL) in Finland. MethodsFinnish Cancer Registry data by histological diagnosis and tumor location (2007-2017) for cases with diffuse large B-cell lymphoma.ResultsDuring 2007–2017, 416 new cases of PCNSL were reported (202 males, 214 females). The average age-adjusted incidence in males vs. females for 2007-2009, 2010-2012, 2013-2015 and 2016-2017 was 0.57/100,000 vs. 0.48/100,000 person-years, 0.66/100,000 vs. 0.55/100,000, 0.53/100,000 vs. 0.63/100,000, and 0.76/100,000 vs. 0.66/100,000 respectively. The incidence was highest, 3.27/100,000, among people aged 75–79 years. From 2007–2012 to 2013–2017, the 2-year age-adjusted survival rate increased from 36% to 41%, and the 4-year survival rates from 31% to 37%. Among patients aged 70+ years, the corresponding 2-year survival rates were 25% and 29%, and the 4-year survival rates 25% and 22%.ConclusionAge-adjusted incidence of PCNSL increased from 0.57/100,000 (2007–2012) to 0.63/100,000 (2013–2017). The annual increase was 3.8% being among the highest reported in the world. The prognosis was poor, although a minor improvement in 4-year survival was found.

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Primary Central Nervous System Lymphoma High Incidence And Poor Survival In Finnish Population-Based Analysis

10.21203/rs.3.rs-1158262/v1 ◽

2021 ◽

Author(s):

Inka Puhakka ◽

Hanne Kuitunen ◽

Pekka Jäkälä ◽

Eila Sonkajärvi ◽

Taina Turpeenniemi-Hujanen ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Survival Rate ◽

Survival Rates ◽

Central Nervous System Lymphoma ◽

Relative Survival ◽

B Cell Lymphoma ◽

Population Based ◽

Incidence Rates ◽

Relative Survival Rate

Abstract Background: We report here the first population-based incidence rates and prognosis of primary central nervous system lymphoma (PCNSL) in Finland. Methods: Finnish Cancer Registry data by histological diagnosis and tumor location (2007-2017) for cases with diffuse large B-cell lymphoma.Results: During 2007–2017, 392 new cases of PCNSL were reported (195 males, 197 females). The average age-adjusted incidence was 0.68/100.000 person-years. Incidence for males was 0.74/100.000 and for females 0.63/100.000, respectively. The incidence was highest, 2.93/100.000, among people aged 75–79 years. Concerning all cases in 2007-2017 the 2-year age-adjusted relative survival rate was 35% and the corresponding 5-year survival rate was 28%. Among patients under the age of 70, the age-adjusted 5-year relative survival rate increased from 36% in 2007-2012 to 43% for 2013-2017. Among patients aged 70+ the corresponding survival rates were poor, 7% and 9%.Conclusions: PCNSL incidence in Finland is among the highest reported in the world. The annual increase in incidence was 2.4%. The prognosis is still dismal, especially in elderly patients.

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Changing Incidence and Survival of Primary Central Nervous System Lymphoma in Australia: A 33-Year National Population-Based Study

Cancers ◽

10.3390/cancers13030403 ◽

2021 ◽

Vol 13 (3) ◽

pp. 403

Author(s):

Alexandra L. Farrall ◽

Justine R. Smith

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Confidence Interval ◽

Median Survival ◽

Survival Probability ◽

Central Nervous System Lymphoma ◽

B Cell Lymphoma ◽

Population Based ◽

Incidence Rates ◽

Cns Lymphoma

Primary central nervous system lymphoma (PCNSL) is a rare brain cancer that remains challenging to study. Epidemiology of PCNSL in the Australian population, which is racially and ethnically diverse, has not been examined previously. Using ICD-O-3.1 Morphology and Topography Codes to identify cases, we analyzed complete datasets from the comprehensive Australian Cancer Database (1982–2014, adults aged ≥ 20 years) to establish incidence rates and trends of PCNSL, and to define survival outcomes of individuals diagnosed with PCNSL, including the predominant diffuse large B-cell lymphoma (DLBCL) type. Age-standardized incidence of PCNSL increased by an average annual 6.8% percent over the study period, with current incidence of 0.43 (95% confidence interval, 0.41–0.46) per 100,000 person-years, in comparison to 21.89 (21.41–22.38) per 100,000 person-years for non-CNS lymphoma. Increase in incidence was characterized by an acute rise between 1996 and 1999, was more pronounced with increasing age, and was driven by increasing incidence of DLBCL. Overall survival for persons diagnosed with PCNSL improved significantly across the study period, with 5-year survival probability increasing from 0.21 (95% confidence interval, 0.16–0.26) to 0.33 (0.30–0.36), and median survival increasing from 318 to 600 days, between 1982–1999 and 2000–2014. Increase in survival was significantly higher for persons with DLBCL versus non-DLBCL PCNSL, but substantially lower than that for persons with non-CNS lymphoma, who had a 5-year survival probability of 0.62 (0.62–0.62) and a median survival of 3388 days in 2000–2014. This study links increasing incidence of PCNSL in Australia to increasing incidence of DLCBL, including in younger adults, and highlights the improving, but low, survival outcome of this cancer.

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Decreasing incidence rates of primary central nervous system lymphoma

Cancer ◽

10.1002/cncr.10643 ◽

2002 ◽

Vol 95 (1) ◽

pp. 193-202 ◽

Cited By ~ 139

Author(s):

Nina S. Kadan-Lottick ◽

Maria C. Skluzacek ◽

James G. Gurney

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Incidence Rates

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Population-Based Incidence and Outcome of Primary Central Nervous System Lymphoma – a Study By the Austrian Brain Tumor Registry, 2005–2010

Annals of Oncology ◽

10.1093/annonc/mdu330.23 ◽

2014 ◽

Vol 25 ◽

pp. iv144

Author(s):

A. Woehrer ◽

M. Hackl ◽

T. Waldhör ◽

J. Hainfellner ◽

A.S. Berghoff ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Brain Tumor ◽

Central Nervous System Lymphoma ◽

Population Based ◽

Tumor Registry

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Conditional relative survival in primary central nervous system lymphoma: a population-based study in the Netherlands

Neuro-Oncology Advances ◽

10.1093/noajnl/vdaa133 ◽

2020 ◽

Vol 2 (1) ◽

Author(s):

Avinash G Dinmohamed ◽

Matthijs van der Meulen ◽

Otto Visser ◽

Jeanette K Doorduijn ◽

Jacoline E C Bromberg

Keyword(s):

Central Nervous System ◽

Nervous System ◽

The Netherlands ◽

Central Nervous System Lymphoma ◽

Relative Survival ◽

Population Based ◽

Population Based Study

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Nomograms for predicting cancer-specific survival in patients with primary central nervous system lymphoma: a population- based analysis

Annals of Translational Medicine ◽

10.21037/atm-21-753 ◽

2021 ◽

Vol 0 (0) ◽

pp. 0-0

Author(s):

Chuanwei Yang ◽

Xiaohui Ren ◽

Yong Cui ◽

Haihui Jiang ◽

Kefu Yu ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Central Nervous System Lymphoma ◽

Population Based ◽

Cancer Specific Survival

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Nonglial Central Nervous System Tumors

10.1093/med/9780197512166.003.0104 ◽

2021 ◽

pp. 931-948

Author(s):

Akanksha Sharma ◽

Alyx B. Porter

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Highly Active Antiretroviral Therapy ◽

Central Nervous System Lymphoma ◽

Non Hodgkin Lymphoma ◽

Highly Active ◽

Acquired Immunodeficiency ◽

Immunodeficiency Syndrome ◽

Adjusted Incidence ◽

The Brain

Primary central nervous system lymphoma (PCNSL) is a rare variant of extranodal non-Hodgkin lymphoma that may involve the brain, leptomeninges, eyes, or spinal cord and accounts for up to 5% of all adult primary brain neoplasms. Age-adjusted incidence has increased in the past 3 decades. Infection with HIV and acquired immunodeficiency syndrome increase the risk of PCNSL by 3,600-fold. However, with highly active antiretroviral therapy, the frequency of immune system compromise sufficient for HIV-associated PCNSL (CD4 count ≤50) is dramatically reduced, along with the risk of this disorder.

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