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

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Rare cancers

Incidence < 6/100,000/EU population

Rare adult solid ca

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Prevalence

Incidence

diecured

Rare adult solid ca

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Rare adult solid ca

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Epidemiology of rare cancers = networks of population-based cancer registries

RARECAREnetwww.rarecarenet.eu94 cancer registries

24 countries

Rare adult solid ca

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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

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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

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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

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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

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Search tool for on line analysis

Rare adult solid ca

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Rare cancers: incidence by countryage-standardised rates

Source: RARECAREnet new results, 2000-07, unpublishedRare adult solid ca

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Rare cancers: five-year survival by countrycase mix adjusted

Source: RARECAREnet new results, 2000-07, unpublishedRare adult solid ca

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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

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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

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• 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

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