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IMPROVED SURVIVAL IN PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA (PCNSL) UP TO AGE 70 ONLY: A POPULATION-BASED STUDY ON INCIDENCE, PRIMARY TREATMENT AND SURVIVAL IN THE NETHERLANDS, 1989-2015
Author(s): ,
Avinash Dinmohamed
Affiliations:
Research,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands;Public Health,Erasmus Univeristy Medical Center,Rotterdam,Netherlands;Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
,
Matthijs van der Meulen
Affiliations:
Neuro-oncology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
,
Otto Visser
Affiliations:
Registration,Netherlands Comprehensive Cancer Organisation (IKNL),Utrecht,Netherlands
,
Jeanette Doorduijn
Affiliations:
Hematology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
Jacoline Bromberg
Affiliations:
Neuro-oncology,Erasmus MC Cancer Institute,Rotterdam,Netherlands
(Abstract release date: 05/18/17) EHA Library. Dinmohamed A. 06/23/17; 181506; P219
Dr. Avinash Dinmohamed
Dr. Avinash Dinmohamed
Contributions
Abstract

Abstract: P219

Type: Poster Presentation

Presentation during EHA22: On Friday, June 23, 2017 from 17:15 - 18:45

Location: Poster area (Hall 7)

Background

PCNSL is a rare, aggressive form of an extranodal non-Hodgkin lymphoma that exclusively affects the CNS. Recent findings from the few available prospective studies demonstrated improved outcome in PCNSL. However, the results from such studies are inherent to patient selection. Population-based studies that assess long-term patterns of incidence, treatment and survival in PCNSL are virtually lacking.

Aims

The aim of this comprehensive nationwide population-based study was to assess trends in incidence, primary treatment and survival among adult PCNSL patients (pts) diagnosed during a 27-year period in the Netherlands.

Methods

We selected all adult (≥18 years) pts diagnosed with PCNSL of the diffuse large B-cell type in the Netherlands between 1989-2015 from the nationwide Netherlands Cancer Registry with survival follow-up through February, 2016. Pts diagnosed without pathological or cytological confirmation (n=50) and pts diagnosed at autopsy were excluded (n=32). Age-standardized incidence rates (ASR) were calculated per 1,000,000 person-years and standardized according to the European standard population. Data on primary treatment (i.e. no therapy, chemotherapy (CT) alone, radiotherapy (RT) alone, and CT+RT) were available for individual pts. Pts were categorized into 4 periods (1989-1994, 1995-2000, 2001-2007 and 2008-2014) and 3 age groups (18-60, 61-70 and >70 years). We calculated relative survival (RS) and the relative excess risk of mortality as measures of disease-specific survival.

Results

We included a total of 1,673 newly diagnosed PCNSL pts in the study (median age, 65 years; age range, 19-89 years; 53% males). The ASR of PCNSL increased from 3.0 in the first period (1989-1995) to 4.4 in the last period (2009-2015), which was consistently higher among males than in females throughout the entire study (4.8 v 4.0 in the last period). The age-specific incidence rates were 2.3, 9.0 and 10.0 in the first period for the three age groups (18-60, 61-70 and >70 years), as compared with 2.7, 18.7 and 19.5 in the last period.
The application of CT+RT increased exclusively among pts age 18-60. More specifically, the proportions for the three age groups were 26, 18 and 4% in the first period, as compared with 60, 10 and 4% in the last period. The use of RT alone among pts age >60 decreased with each period, following the wider use of CT alone over time, especially for pts age 61-70 years. The proportions of CT alone for the three  age groups were 11, 8 and 2% in the first period, as compared with 31, 64 and 32% in the last period. Of note, 38 and 26% of pts age >70 received no therapy and RT alone in the last period, respectively.
Five-year RS only improved for pts age 18-70 (Fig 1). Five-year RS (95% confidence intervals) was 22% (16%>30%), 13% (7%>22%), and 3% (1%>10%) in the first period for the three age groups, as compared with 56% (47%>64%), 35% (28%>43%) and 6% (2%>13%) in the last period. A multivariable survival model confirmed the adverse effect of older age on excess mortality and an improvement of survival over time. However, when information on treatment was added to that model, the effect of period lost statistical significance. This suggest that treatment contributed to the improved survival over time. Older age remained a predictor of poor prognosis.

Conclusion

The incidence of PCNSL steadily increases among pts >60 years, which might in part be related to improved diagnostic practices among the elderly over time. RS increased over the past decades for pts age 70 or below. This is largely explained by the increased use of intensive therapy over time. Although the use of CT alone gradually increased among pts >70 years, their survival is still poor. Therefore, there is an urgent need to design specific trials for elderly PCNSL pts to improve their survival.

Session topic: 20. Aggressive Non-Hodgkin lymphoma - Clinical

Keyword(s): CNS lymphoma, Treatment, Survival, epidemiology

Abstract: P219

Type: Poster Presentation

Presentation during EHA22: On Friday, June 23, 2017 from 17:15 - 18:45

Location: Poster area (Hall 7)

Background

PCNSL is a rare, aggressive form of an extranodal non-Hodgkin lymphoma that exclusively affects the CNS. Recent findings from the few available prospective studies demonstrated improved outcome in PCNSL. However, the results from such studies are inherent to patient selection. Population-based studies that assess long-term patterns of incidence, treatment and survival in PCNSL are virtually lacking.

Aims

The aim of this comprehensive nationwide population-based study was to assess trends in incidence, primary treatment and survival among adult PCNSL patients (pts) diagnosed during a 27-year period in the Netherlands.

Methods

We selected all adult (≥18 years) pts diagnosed with PCNSL of the diffuse large B-cell type in the Netherlands between 1989-2015 from the nationwide Netherlands Cancer Registry with survival follow-up through February, 2016. Pts diagnosed without pathological or cytological confirmation (n=50) and pts diagnosed at autopsy were excluded (n=32). Age-standardized incidence rates (ASR) were calculated per 1,000,000 person-years and standardized according to the European standard population. Data on primary treatment (i.e. no therapy, chemotherapy (CT) alone, radiotherapy (RT) alone, and CT+RT) were available for individual pts. Pts were categorized into 4 periods (1989-1994, 1995-2000, 2001-2007 and 2008-2014) and 3 age groups (18-60, 61-70 and >70 years). We calculated relative survival (RS) and the relative excess risk of mortality as measures of disease-specific survival.

Results

We included a total of 1,673 newly diagnosed PCNSL pts in the study (median age, 65 years; age range, 19-89 years; 53% males). The ASR of PCNSL increased from 3.0 in the first period (1989-1995) to 4.4 in the last period (2009-2015), which was consistently higher among males than in females throughout the entire study (4.8 v 4.0 in the last period). The age-specific incidence rates were 2.3, 9.0 and 10.0 in the first period for the three age groups (18-60, 61-70 and >70 years), as compared with 2.7, 18.7 and 19.5 in the last period.
The application of CT+RT increased exclusively among pts age 18-60. More specifically, the proportions for the three age groups were 26, 18 and 4% in the first period, as compared with 60, 10 and 4% in the last period. The use of RT alone among pts age >60 decreased with each period, following the wider use of CT alone over time, especially for pts age 61-70 years. The proportions of CT alone for the three  age groups were 11, 8 and 2% in the first period, as compared with 31, 64 and 32% in the last period. Of note, 38 and 26% of pts age >70 received no therapy and RT alone in the last period, respectively.
Five-year RS only improved for pts age 18-70 (Fig 1). Five-year RS (95% confidence intervals) was 22% (16%>30%), 13% (7%>22%), and 3% (1%>10%) in the first period for the three age groups, as compared with 56% (47%>64%), 35% (28%>43%) and 6% (2%>13%) in the last period. A multivariable survival model confirmed the adverse effect of older age on excess mortality and an improvement of survival over time. However, when information on treatment was added to that model, the effect of period lost statistical significance. This suggest that treatment contributed to the improved survival over time. Older age remained a predictor of poor prognosis.

Conclusion

The incidence of PCNSL steadily increases among pts >60 years, which might in part be related to improved diagnostic practices among the elderly over time. RS increased over the past decades for pts age 70 or below. This is largely explained by the increased use of intensive therapy over time. Although the use of CT alone gradually increased among pts >70 years, their survival is still poor. Therefore, there is an urgent need to design specific trials for elderly PCNSL pts to improve their survival.

Session topic: 20. Aggressive Non-Hodgkin lymphoma - Clinical

Keyword(s): CNS lymphoma, Treatment, Survival, epidemiology

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