Epidemiology of rare cancers and inequalities in oncologic outcomes

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Abstract

Rare cancers epidemiology is better known compared to the other rare diseases. Thanks to the long history of the European population-based cancer registries and to the EUROCARE huge database, the burden of rare cancers has been estimated the European (EU28) population. A considerable fraction of all cancers is represented by rare cancers (24%). They are a heterogeneous group of diseases, but they share similar problems: uncertainty of diagnosis, lack of therapies, poor research opportunities, difficulties in clinical trials, lack of expertise and of centres of reference. This paper analyses the major epidemiological indicators of frequency (incidence and prevalence) and outcome (5-year survival) of all rare cancers combined and of selected rare cancers that will be in depth treated in this monographic issue. Source of the results is the RARECAREnet search tool, a database publicly available. Disparities both in incidence and survival, and consequently in prevalence of rare cancers were reported across European countries. Major differences were shown in outcome: 5-year relative survival for all rare cancers together, adjusted by age and case-mix, varied from 55% or more (Italy, Germany, Belgium and Iceland) and less than 40% (Bulgaria, Lithuania and Slovakia). Similarly, for all the analyzed rare cancers, a large survival gap was observed between the Eastern and the Nordic and Central European regions. Dramatic geographical variations were assessed for curable cancers like testicular and non epithelial ovarian cancers. Geographical difference in the annual age-adjusted incidence rates for all rare cancers together varied between >140 per 100,000 (Italy, Scotland, France, Germany, and Switzerland) and
Original languageEnglish
JournalEuropean Journal of Surgical Oncology
DOIs
Publication statusPublished - 2017

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Epidemiology
Neoplasms
Italy
Germany
Incidence
Databases
Lithuania
Iceland
Bulgaria
Slovakia
Diagnosis-Related Groups
Belgium
Testicular Neoplasms
Scotland
Rare Diseases
Switzerland
Population
Uncertainty
France
Registries

Keywords

  • Europe
  • Population-based cancer registry
  • Rare cancers

Cite this

@article{5e91786e129f4ba1a59da94e9b8bdaf3,
title = "Epidemiology of rare cancers and inequalities in oncologic outcomes",
abstract = "Rare cancers epidemiology is better known compared to the other rare diseases. Thanks to the long history of the European population-based cancer registries and to the EUROCARE huge database, the burden of rare cancers has been estimated the European (EU28) population. A considerable fraction of all cancers is represented by rare cancers (24{\%}). They are a heterogeneous group of diseases, but they share similar problems: uncertainty of diagnosis, lack of therapies, poor research opportunities, difficulties in clinical trials, lack of expertise and of centres of reference. This paper analyses the major epidemiological indicators of frequency (incidence and prevalence) and outcome (5-year survival) of all rare cancers combined and of selected rare cancers that will be in depth treated in this monographic issue. Source of the results is the RARECAREnet search tool, a database publicly available. Disparities both in incidence and survival, and consequently in prevalence of rare cancers were reported across European countries. Major differences were shown in outcome: 5-year relative survival for all rare cancers together, adjusted by age and case-mix, varied from 55{\%} or more (Italy, Germany, Belgium and Iceland) and less than 40{\%} (Bulgaria, Lithuania and Slovakia). Similarly, for all the analyzed rare cancers, a large survival gap was observed between the Eastern and the Nordic and Central European regions. Dramatic geographical variations were assessed for curable cancers like testicular and non epithelial ovarian cancers. Geographical difference in the annual age-adjusted incidence rates for all rare cancers together varied between >140 per 100,000 (Italy, Scotland, France, Germany, and Switzerland) and",
keywords = "Europe, Population-based cancer registry, Rare cancers",
author = "G. Gatta and A. Trama and R. Capocaccia and group, {RARECAREnet working} and Francisci, {Silvia Giovanna Luisa} and andrea tavilla and Daniela Pierannunzio and Silvia Rossi and Mariano Santaquilani and Arnold Knijn",
note = "Export Date: 12 April 2018 Article in Press CODEN: EJSOE Correspondence Address: Gatta, G.email: gemma.gatta@istitutotumori.mi.it Funding details: 2000111201, Chafea, Consumers, Health, Agriculture and Food Executive Agency Funding details: EC, European Commission Funding text: This research was funded by the European Commission through the Consumers, Health, Agriculture and Food Executive Agency (Chafea) ; Grant No. 2000111201 ; Information network on rare cancers—RARECARENet. We also recognize the private donation provided by friends and family of Gabriele Guarneri for rare cancer epidemiological research.",
year = "2017",
doi = "10.1016/j.ejso.2017.08.018",
language = "English",
journal = "European Journal of Surgical Oncology",
issn = "0748-7983",
publisher = "W.B. Saunders Ltd",

}

TY - JOUR

T1 - Epidemiology of rare cancers and inequalities in oncologic outcomes

AU - Gatta, G.

AU - Trama, A.

AU - Capocaccia, R.

AU - group, RARECAREnet working

AU - Francisci, Silvia Giovanna Luisa

AU - tavilla, andrea

AU - Pierannunzio, Daniela

AU - Rossi, Silvia

AU - Santaquilani, Mariano

AU - Knijn, Arnold

N1 - Export Date: 12 April 2018 Article in Press CODEN: EJSOE Correspondence Address: Gatta, G.email: gemma.gatta@istitutotumori.mi.it Funding details: 2000111201, Chafea, Consumers, Health, Agriculture and Food Executive Agency Funding details: EC, European Commission Funding text: This research was funded by the European Commission through the Consumers, Health, Agriculture and Food Executive Agency (Chafea) ; Grant No. 2000111201 ; Information network on rare cancers—RARECARENet. We also recognize the private donation provided by friends and family of Gabriele Guarneri for rare cancer epidemiological research.

PY - 2017

Y1 - 2017

N2 - Rare cancers epidemiology is better known compared to the other rare diseases. Thanks to the long history of the European population-based cancer registries and to the EUROCARE huge database, the burden of rare cancers has been estimated the European (EU28) population. A considerable fraction of all cancers is represented by rare cancers (24%). They are a heterogeneous group of diseases, but they share similar problems: uncertainty of diagnosis, lack of therapies, poor research opportunities, difficulties in clinical trials, lack of expertise and of centres of reference. This paper analyses the major epidemiological indicators of frequency (incidence and prevalence) and outcome (5-year survival) of all rare cancers combined and of selected rare cancers that will be in depth treated in this monographic issue. Source of the results is the RARECAREnet search tool, a database publicly available. Disparities both in incidence and survival, and consequently in prevalence of rare cancers were reported across European countries. Major differences were shown in outcome: 5-year relative survival for all rare cancers together, adjusted by age and case-mix, varied from 55% or more (Italy, Germany, Belgium and Iceland) and less than 40% (Bulgaria, Lithuania and Slovakia). Similarly, for all the analyzed rare cancers, a large survival gap was observed between the Eastern and the Nordic and Central European regions. Dramatic geographical variations were assessed for curable cancers like testicular and non epithelial ovarian cancers. Geographical difference in the annual age-adjusted incidence rates for all rare cancers together varied between >140 per 100,000 (Italy, Scotland, France, Germany, and Switzerland) and

AB - Rare cancers epidemiology is better known compared to the other rare diseases. Thanks to the long history of the European population-based cancer registries and to the EUROCARE huge database, the burden of rare cancers has been estimated the European (EU28) population. A considerable fraction of all cancers is represented by rare cancers (24%). They are a heterogeneous group of diseases, but they share similar problems: uncertainty of diagnosis, lack of therapies, poor research opportunities, difficulties in clinical trials, lack of expertise and of centres of reference. This paper analyses the major epidemiological indicators of frequency (incidence and prevalence) and outcome (5-year survival) of all rare cancers combined and of selected rare cancers that will be in depth treated in this monographic issue. Source of the results is the RARECAREnet search tool, a database publicly available. Disparities both in incidence and survival, and consequently in prevalence of rare cancers were reported across European countries. Major differences were shown in outcome: 5-year relative survival for all rare cancers together, adjusted by age and case-mix, varied from 55% or more (Italy, Germany, Belgium and Iceland) and less than 40% (Bulgaria, Lithuania and Slovakia). Similarly, for all the analyzed rare cancers, a large survival gap was observed between the Eastern and the Nordic and Central European regions. Dramatic geographical variations were assessed for curable cancers like testicular and non epithelial ovarian cancers. Geographical difference in the annual age-adjusted incidence rates for all rare cancers together varied between >140 per 100,000 (Italy, Scotland, France, Germany, and Switzerland) and

KW - Europe

KW - Population-based cancer registry

KW - Rare cancers

U2 - 10.1016/j.ejso.2017.08.018

DO - 10.1016/j.ejso.2017.08.018

M3 - Article

JO - European Journal of Surgical Oncology

JF - European Journal of Surgical Oncology

SN - 0748-7983

ER -