Rare cancers are not so rare: the rare cancer burden in Europe.
OBJECTIVE
Epidemiologic information on rare cancers is scarce. The project Surveillance of Rare Cancers in Europe (RARECARE) provides estimates of the incidence, prevalence and survival of rare cancers in Europe based on a new and comprehensive list of these diseases.


METHODS
RARECARE analysed population-based cancer registry (CR) data on European patients diagnosed from 1988 to 2002, with vital status information available up to 31st December 2003 (latest date for which most CRs had verified data). The mean population covered was about 162,000,000. Cancer incidence and survival rates for 1995-2002 and prevalence at 1st January 2003 were estimated.


RESULTS
Based on the RARECARE definition (incidence <6/100,000/year), the estimated annual incidence rate of all rare cancers in Europe was about 108 per 100,000, corresponding to 541,000 new diagnoses annually or 22% of all cancer diagnoses. Five-year relative survival was on average worse for rare cancers (47%) than common cancers (65%). About 4,300,000 patients are living today in the European Union with a diagnosis of a rare cancer, 24% of the total cancer prevalence.


CONCLUSIONS
Our estimates of the rare cancer burden in Europe provide the first indication of the size of the public health problem due to these diseases and constitute a useful base for further research. Centres of excellence for rare cancers or groups of rare cancers could provide the necessary organisational structure and critical mass for carrying out clinical trials and developing alternative approaches to clinical experimentation for these cancers.
Department of Preventive and Predictive Medicine, Fondazione IRCSS, Istituto Nazionale dei Tumori, Via Venezian 1, Milan, Italy. gemma.gatta@istitutotumori.mi.it
Gemma
G
Gatta
Jan Maarten
JM
van der Zwan
Paolo G
PG
Casali
Sabine
S
Siesling
Angelo Paolo
AP
Dei Tos
Ian
I
Kunkler
Renée
R
Otter
Lisa
L
Licitra
Sandra
S
Mallone
Andrea
A
Tavilla
Annalisa
A
Trama
Riccardo
R
Capocaccia
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000453:epidemiology*; D015995:Prevalence; D035583:Rare Diseases / Q000453:epidemiology*; D012042:Registries; D016019:Survival Analysis; D055815:Young Adult