Racial differences in primary central nervous system lymphoma incidence and survival rates

Primary central nervous system lymphoma (PCNSL) is a rare and aggressive non-Hodgkin lymphoma that affects the brain, eyes, cerebrospinal fluid (CSF), or spinal cord without systemic involvement. Here, we review the clinical presentation, diagnostic work-up, novel pathophysiologic insights, and treatment of immunocompetent PCNSL patients. Diagnosis of PCNSL requires a high level of suspicion as clinical signs and deficits can vary depending upon the involved CNS compartments. Rapid initiation of therapy is essential for good neurologic recovery and disease control. In general, the prognosis of PCNSL has improved significantly over the past few decades, largely due to the introduction and wide-spread use of high-dose methotrexate (MTX) chemotherapy, considered the backbone of first-line polychemotherapy treatment. Upon completion of MTX-based treatment, a consolidation strategy is often required and can consist of non-myeloablative or myeloablative chemotherapy followed by autologous stem cell transplant, radiation, maintenance therapy, or observation. Unfortunately, relapse is common and 5-year survival rates stand at only 30-40%. Novel insights into the pathophysiology of PCNSL have identified key mechanisms in tumor pathogenesis including activation of the B-cell receptor pathway, a suppressed tumor immune microenvironment, and immune evasion. These insights have led to the identification of novel small molecules and agents targeting these aberrant pathways. Agents such as the Bruton Tyrosine Kinase (BTK) inhibitor ibrutinib or immunomodulatory drugs (IMiDs) like lenalidomide or pomalidomide have shown promising response rates in the clinical trial setting for recurrent/refractory PCNSL and are increasingly being adopted in clinical use.

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

10.21203/rs.3.rs-231278/v1 ◽

2021 ◽

Author(s):

Inka Puhakka ◽

Outi Kuittinen ◽

Pekka Jäkälä ◽

Hanne Kuitunen ◽

Taina Turpeenniemi-Hujanen ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Survival Rates ◽

Central Nervous System Lymphoma ◽

Tumor Location ◽

Population Based ◽

Incidence Rates ◽

Cancer Registry Data ◽

A Minor ◽

Adjusted Incidence

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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