Information network on rare cancers

INFORMATION NETWORK ON RARE CANCERS (RARECARENet)Kick-off meeting - Friday, July 13 2012

Gemma GattaFondazione IRCCS, Istituto Nazionale dei Tumori – Milan (Italy)

Information network on rare cancers

to discuss the project among all the project partners

the Work Package (WP) leaders have been invited:– to describe objectives and activities of

their WPs– to provide a detailed planning of WP

activities

Objectives of the kick-off meeting

Information network on rare cancers

INFORMATION NETWORK ON RARE CANCERS AGENDA

Introduction 10:30 Welcome and objectives of the meeting

G Gatta 10:40 The background (from RARECARE to RARECARENet) G Gatta 11:00 Overview of the RARECARENet project

A Trama 11:20 Discussion Work packages presentations and discussion 11:30 WP 4 Information on epidemiology of rare cancers R Capocaccia/S Mallone 12:00 WP5 Information on centres of expertise for rare cancers S Siesling 12:30 WP6 Information on clinical management of rare cancers L Licitra 13:00 Lunch break 13:45 WP7 Information for patients with rare cancers F De Lorenzo/ J Pelouchová 14:15 WP2 Dissemination

I Kunkler 14:45 WP3 Evaluation G Gatta (on behalf of E Benamou) 15:30 Coffee break 15:45 Practical issues related to RARECARENet implementation

H Mileva/A Trama 16:30 AOB 17:00 Closing remarks

Information network on rare cancers

What I am expect from this meeting

Suggestions and comments Identification of possible problems with

solutions Planning of the interaction between WPs

Background: from RARECARE to RARECARENet

Why rare cancers?

Information network on rare cancers

EUROCARE

About 100 European cancer registries (in 22 EU Countries)

www.eurocare.it

Information network on rare cancers

Survival of adult patients with rare cancer in Europe (Europe against Cancer Programme) with the ENCR (2001-2003)

Two unsuccesful FP6 proposals

BEFORE RARECARE

Information network on rare cancers

Several papers on rare cancer survival

Several papers on rare cancer survival

Several papers on rare cancer survival

Several papers on rare cancer survival

Information network on rare cancers

RARECARE (2007-2010)

Aims

To provide a definition of “rare cancers” and a list of cancers

To estimate the burden of rare cancers in Europe To improve the quality of data in cancer registration To develop strategies for the diffusion of information

among all the key players

Essential source of data = population-based cancer registries

RARE DISEASES

Information network on rare cancers

– Clinical vs site cancer entities– ICD-O topography and morphology codes– About 220 tumour entities, 186 rare– Threshold: incidence vs prevalence

DEFINITION AND LIST OF RARE CANCERS

Information network on rare cancers

Incidence vs prevalence

Information network on rare cancers

All rare cancers, but cervix uteri and thyroid carcinoma are also rare according to the European definition

Adenocarcinoma and squamous cell carcinoma of lung, adenocarcinoma of stomach are rare according to the European definition and rare according to the RARECARE definition

Incidence vs prevalence

Information network on rare cancers

Complete prevalence

Observed prevalence + completeness index

N C = NO(L)+ NU(L)

4 models per each entity (around 1000 different models)

Prevalence is not an easy task

Information network on rare cancers

Threshold: issuesThreshold: issues

How many cancer diagnoses refer to rare cancers?

Does rarity affect the possibility to carry out effective research?

Is clinical decision making more difficult?

Threshold for rarity < 6/100.000/year

Information network on rare cancers

Cut point: too highCut point: too high

Threshold of <3/100,000/year instead of <6/100,000/year glial CNS tumours, epithelial cancers of the oral cavity epithelial cancers of gallbladder soft tissue sarcomas tumours of testis carcinomas of endocrine organs Myeloproliferative neoplasms Acute myeloid leukaemia,

Information network on rare cancers

0

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Incid

ence

rate

Countries

Crude incidence of rare cancers by country

Variation depends on:•Risk factors•Population age•Case mix •Availability of expertise•...

In Europe 97/100,000/year

Information network on rare cancers

Incidence Incidence rates for rare rates for rare and common cancersand common cancers

AGEAGE

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00-04 05-09 10-14 15-19 20-24 25-29 30-34 35-39 40-44 45-49 50-54 55-59 60-64 65-69 70-74 75-79 80-84 85+

Common

Rare

Information network on rare cancers

All rare cancers All rare cancers by countrycountryHow many new diagnoses/yearHow many new diagnoses/year

Iceland 330

Malta 370

Luxembourg 550

Cyprus 870

Estonia 1500

Slovenia 2000

Latvia 2500

Ireland 3700

Slovakia 4500

Norway 4800

Croatia 4900

Finland 5800

Denmark 6000

Austria 8000

Bulgaria 8200

Switzerland 9700

Sweden 10400

Portugal 10500

Hungary 10900

Czech Rep 11500

Belgium 11800

Greece 12100

Netherlands 17800

Romania 23300

Poland 30500

Spain 51900

UK_England 68000

Italy 80500

France 81000

Germany 117200

EUROPE 27 539000

Information network on rare cancers

adrenal cancersadrenal cancers by by countrycountryHow many new diagnoses/yearHow many new diagnoses/yearIceland 1Malta 1Luxembourg 1Cyprus 1Estonia 2Slovenia 4Latvia 4Ireland 8Croatia 8Norway 9Finland 10Slovakia 10Denmark 10Bulgaria 14Switzerland 14Austria 15

Sweden 17Hungary 18Czech Rep 19Belgium 19Greece 20Portugal 20Netherlands 30Romania 39Poland 69Spain 83Italy 109UK_England 112France 116Germany 147EUROPE 895

Information network on rare cancers

INCIDENCE Distribution of :

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Number of cancer types Annual number of diagnoses

Information network on rare cancers

Five-year survival of rare and Five-year survival of rare and common cancers, by agecommon cancers, by age

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0-39 40-59 60-74 75+ All ages

Common

Rare

5-year survival: common cancers 64% 5-year survival: common cancers 64% vsvs rare cancers 48% rare cancers 48%

Information network on rare cancers

Five-year survival (%) by European countrycountry commoncommon rarerare

0 10 20 30 40 50 60 70 80

Slovakia

Slovenia

Poland

UK_Wales

Malta

Netherlands

Spain

UK_England

Portugal

Belgium

France

Iceland

Switzerland

EUROPE

Austria

Sweden

UK_Scotland

Germany

Italy

Norway

UK_Northern Ireland

Ireland

% 0 10 20 30 40 50 60 70 80

Slovakia

UK_Wales

France

UK_England

UK_Scotland

Slovenia

Netherlands

Poland

Switzerland

EUROPE

Portugal

Ireland

Belgium

Germany

Spain

UK_Northern Ireland

Austria

Norway

Italy

Sweden

Malta

Iceland

%

Information network on rare cancers

Survival variation between European regions

Information network on rare cancers

Survival variation between European regions

Information network on rare cancers

Rare cancers burdenRare cancers burden

Incidence = 500,000 new diagnosis/year

22% of all cancer diagnoses/yr

Prevalence = 4 million patients 24% of the total cancer

prevalence Survival = common cancers 64% vs rare cancers 48%

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Information network on rare cancers

DisseminationDissemination

www.rarecare.eu

Information network on rare cancers

DisseminationDisseminationPapers in peer review journalsPapers in peer review journals

Information network on rare cancers

RARECARE – WHAT’S ADDED

Put numbers to a problem long known to exist

Show low outcome of rare cancers

Made prevalence available for rare cancers

Identified a new priority for population-based cancer registries

Information network on rare cancers

RARECARE: http://www.rarecare.eu/

Enjoy the meeting!Enjoy the meeting!