1. Information network on rare cancers INFORMATION NETWORK ON RARE CANCERS (RARECARENet) Kick-off meeting - Friday, July 13 2012 Gemma Gatta Fondazione IRCCS, Istituto Nazionale dei Tumori – Milan (Italy)

2. 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

3. Information network on rare cancers INFORMATION NETWORK ON RARE CANCERS AGENDA

4. 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

5. Background: from RARECARE to RARECARENet

6. Why rare cancers?

7. Information network on rare cancers EUROCARE About 100 European cancer registries (in 22 EU Countries) www.eurocare.it

8. 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 B EFORE RARECARE

9. Information network on rare cancers Several papers on rare cancer survival

13. 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

14. 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

15. Information network on rare cancers Incidence vs prevalence

16. 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

17. Information network on rare cancers Complete prevalence Observed prevalence + completeness index N C = N O (L)+ N U (L) 4 models per each entity (around 1000 different models) Prevalence is not an easy task

18. Information network on rare cancers Threshold: 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

19. Information network on rare cancers Cut 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,

20. Information network on rare cancers Variation depends on: Risk factors Population age Case mix Availability of expertise... In Europe 97/100,000/year

21. Information network on rare cancers Incidence rates for rare and common cancers AGE

22. Information network on rare cancers All rare cancers country How many new diagnoses/year All rare cancers by country How many new diagnoses/year Iceland330 Malta370 Luxembourg550 Cyprus870 Estonia1500 Slovenia2000 Latvia2500 Ireland3700 Slovakia4500 Norway4800 Croatia4900 Finland5800 Denmark6000 Austria8000 Bulgaria8200 Switzerland9700 Sweden10400 Portugal10500 Hungary10900 Czech Rep11500 Belgium11800 Greece12100 Netherlands17800 Romania23300 Poland30500 Spain51900 UK_England68000 Italy80500 France81000 Germany117200 EUROPE 27539000

23. Information network on rare cancers adrenal cancers by country How many new diagnoses/year

24. Information network on rare cancers INCIDENCE Distribution of : Number of cancer types Annual number of diagnoses

25. Information network on rare cancers Five-year survival of rare and common cancers, by age 5-year survival: common cancers 64% vs rare cancers 48%

26. Information network on rare cancers country commonrare Five-year survival (%) by European country common rare

27. Information network on rare cancers Survival variation between European regions

28. Information network on rare cancers Survival variation between European regions

29. Information network on rare cancers Rare 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%

30. Information network on rare cancers Dissemination

31. www.rarecare.eu

32. Information network on rare cancers Dissemination Papers in peer review journals

33. Information network on rare cancers Dissemination, papers in peer review journals Rare Cancers of head and neck Incidence, prevalence and survival in rare epithelial digestive cancers Rare thoracic cancers, including peritoneum mesothelioma Descriptive Epidemiology of Sarcomas in Europe. Report from the RARECARE Project Extracutaneous melanomas (uveal and mucosal) and adnexal skin carcinomas in Europe Rare cancers of female genital tract Rare tumours of testis and extragonadal germ cell tumours Rare Urogenital Cancers Rare Central Nervous System Cancers Endocrine Tumours: results of the RARECARE project Neuroendocrine Tumours: results of the RARECARE project The embryonal cancers in Europe Rare Myeloid malignancies

34. 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

35. Information network on rare cancers Further prospectives RARECAREnet To describe diagnosis and treatment pathway for across EU countries from registry data. To identify the qualification criteria for centres of expertise and list centres of expertise To produce and disseminate information on diagnosis and management of rare cancers To develop a clinical database on very rare cancers to develop new knowledge on their clinical management

36. Information network on rare cancers RARECARE: http://www.rarecare.eu/http://www.rarecare.eu/ Enjoy the meeting!