ESO-ESMO-RCE Clinical Update on Rare Adult Solid Cancers
25/11/2016 - 27/11/2016, Milan, Italy
The epidemiology of rare cancersAnnalisa Trama
Fondazione IRCCS Istituto Nazionale Tumori, Milan - Italy
Rare adult solid ca
ncers
Rare cancers
Incidence < 6/100,000/EU population
Rare adult solid ca
ncers
Prevalence
Incidence
diecured
Rare adult solid ca
ncers
Rare adult solid ca
ncers
Epidemiology of rare cancers = networks of population-based cancer registries
RARECAREnetwww.rarecarenet.eu94 cancer registries
24 countries
Rare adult solid ca
ncers
List of rare cancers
198 rare cancers www.rarecarenet.eu
12 families of rare cancers1. Pediatric cancers
2. Haematologic rare neoplasms
3. Sarcomas4. Rare thoracic cancers5. Neuroendocrine tumours 6. Head & neck cancers7. Central nervous system tumours 8. Rare female genital cancers9. Rare urological and male genital tumours10. Endocrine gland tumours11. Digestive rare cancers12. Rare skin cancers & non-cutaneous melanoma
Rare adult solid ca
ncers
The burden of rare cancers
N=640,000
24%
% rare cancers on new diagnosis
Germany 22
France 28
Italy 20
Spain 26
Poland 21
Netherlands 25
Belgium 23
Portugal 25
Austria 22
Bulgaria 21
Finland 19
Slovakia 25
Ireland 17
Croatia 21
Lithuania 22
Slovenia 23
Rare adult solid ca
ncers
Rare cancers familiesRare thoracic cancers Crude Incidence Rate
per 100,000 per yearEstimated new cases
at 2013 in EU (28)Five-year relative
survival (%)
Trachea 0.11 574 18.0Thymus 0.18 905 64Malignant mesothelioma 2.14 10,868 5Rare tumours of lung 4.37 24,930 15
Endocrine glandtumours
Crude Incidence Rate per 100,000 per year
Estimated new cases at 2013 in EU (28)
Five-year relative survival (%)
Pituitary gland 0.04 206 64
Thyroid gland 5.07 26,768 91Parathyroid gland 0.03 143 81Adrenal gland 0.22 1,205 32
Rare adult solid ca
ncers
Family
Crude Incidence Rate per 100,000 per
year
Estimated new cases at 2013 in
EU (28)
Five-year relative
survival (%)
Head and neck cancers 16.82 84,989 52.1
Digestive rare cancers 21.94 112,351 15.3
Thoracic rare cancers 6.80 37,277 13.4
Female genital rare cancers 22.73 113,796 57.7
Male genital and urogenital rare cancers 7.09 38,138 73.64
Neuroendocrine tumours 3.51 19587 53.5
Cancers of the endocrine organs 5.35 28,322 88.08
Sarcomas 5.86 31,916 59.53
Cancers of the central nervous system (CNS) 7.56 36,343 21.3
Rare skin cancers and non-cutaneous melanoma 1.22 7,086 70.2
Embrional tumours 0.34 1,822 78.6
Haematological rare malignancies 27.73 156,099 50.5
Rare cancers families
Rare adult solid ca
ncers
Search tool for on line analysis
Rare adult solid ca
ncers
Rare cancers: incidence by countryage-standardised rates
Source: RARECAREnet new results, 2000-07, unpublishedRare adult solid ca
ncers
Rare cancers: five-year survival by countrycase mix adjusted
Source: RARECAREnet new results, 2000-07, unpublishedRare adult solid ca
ncers
What kind of health care organisation?
Country Population Incidence rate
0.5/100,000 (nasal cavity cancers)
1/100,000 (anal canal cancers)
5/100,000 (soft tissue sarcomas)
France 65,578,819 328 656 3,935
Italy 59,685,227 298 597 3,581
Poland 38,533,299 193 385 2,312
Netherlands 16,779,575 84 168 1,007
Belgium 11,161,642 56 112 670
Portugal 10,487,289 52 105 629
Bulgaria 7,284,552 36 73 437
Finland 5,426,674 27 54 326
Slovakia 5,410,836 27 54 325
Slovenia 2,058,821 10 21 124
Estonia 1,320,174 7 13 79
Rare adult solid ca
ncers
Rare cancers 5-year relative survival overtime trends
-100
-80
-60
-40
-20
0
20
40
60
80
100
0,0 0,3 2,5
Surv
ival
dif
fere
nce
precision
Rare adult solid ca
ncers
• Rare cancers definition is based on incidence
• Rare cancers are the same in each country because the definition is based on the EU population not the country specific ones
• Rare cancers burden is high in EU and is each EU country
• Rare cancers are 198 and are heterogeneus in terms of incidence and survival
• Incidence differ accross countries (different case-mix of cancers, quality of pathologicaldiagnosis, quality of registration, different risk factors)
• Survival differ accross countries (different case-mix of cancers, biology of the cancer, healthcare organisation)
• Survival is low for many rare cancers and it is not increasing (with few exceptions)
Thank you for your attention!
Rare adult solid ca
ncers