Solid Cancers: An Introduction - Slide 1 - G. Gatta - Rare cancers: frequency and survival

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Solid Cancers: An Introduction - Slide 1 - G. Gatta - Rare cancers: frequency and survival

  1. 1. Rare cancers: frequency and survival Gemma Gatta Fondazione IRCCS "Istituto Nazionale dei Tumori", Milan–Italy
  2. 2. This project is supported by the Executive Agency for Health and Consumers (EAHC) of the European Commission
  3. 3. <ul><li>Aims </li></ul><ul><li>To provide a definition of “rare cancers” and a list of cancers </li></ul><ul><li>To estimate the burden of rare cancers in Europe </li></ul><ul><li>To improve the quality of data in cancer registration </li></ul><ul><li>To develop strategies for the diffusion of information among all the key players </li></ul><ul><li>Duration: 3 years (starting date 1 st April 2007) </li></ul>Surveillance of rare cancers in Europe
  4. 4. <ul><li>89 European cancer registries (in 21 EU countries) </li></ul><ul><li>Population covered: </li></ul><ul><li>162 millions </li></ul><ul><li>Selected diagnosis period: 1995-2002 </li></ul><ul><li>Malignant cases: 4 millions </li></ul>European coverage
  5. 5. <ul><li>Rationale </li></ul><ul><ul><li>Frequency </li></ul></ul><ul><ul><ul><li>Rare cancers present specific problems in </li></ul></ul></ul><ul><ul><ul><li>clinical decision making </li></ul></ul></ul><ul><ul><ul><li>health care organization </li></ul></ul></ul><ul><ul><ul><li>clinical research </li></ul></ul></ul><ul><ul><ul><li>because of their low frequency </li></ul></ul></ul>Operational definition of “rare cancer”
  6. 6. Indicators <ul><li>Frequency </li></ul><ul><li>Incidence </li></ul><ul><ul><li>Period of diagnosis 1995-2002 </li></ul></ul><ul><li>Prevalence </li></ul><ul><ul><li>index date of 1 st January 2003 </li></ul></ul><ul><li>Outcome </li></ul><ul><li>Survival </li></ul><ul><ul><li>Period of diagnosis 1995-1999 </li></ul></ul><ul><ul><li>( followed-up at least until the end of 2003) </li></ul></ul>
  7. 7. Indicators <ul><li>Frequency </li></ul><ul><li>Incidence </li></ul><ul><ul><li>How many new diagnoses in a year </li></ul></ul><ul><li>Prevalence </li></ul><ul><li>How many alive cases (new and old diagnoses) </li></ul><ul><li>Outcome </li></ul><ul><li>Survival </li></ul><ul><ul><li>How many, in % , patients survive at 1,2,3,5, … years since diagnosis </li></ul></ul>
  8. 8. Cut point: issues <ul><li>How many cancer diagnoses refer to rare cancers? </li></ul><ul><li>Does rarity affect the possibility to carry out effective research? </li></ul><ul><li>Is clinical decision making more difficult? </li></ul><ul><ul><li>Threshold for rarity < 6/100.000/year </li></ul></ul>
  9. 9. Rare cancers definition <ul><li>Incidence is the best indicator to define rare cancers </li></ul><ul><li>Prevalence <50x100,000 (rare) </li></ul><ul><li>squamous cell ca of the lung </li></ul><ul><li>adenocarcinoma of the lung </li></ul><ul><li>adenocarcinoma of the stomach </li></ul>Incidence >6/100,000 (common)
  10. 10. <ul><li>Grouped into </li></ul><ul><li>morphologic+ </li></ul><ul><li>topographic entities </li></ul><ul><li>Clinical entities </li></ul><ul><li>Group of experts, </li></ul><ul><li>consensus process </li></ul>
  11. 11. The list of cancers http://www.rarecare.eu 194 rare cancers
  12. 12. incidence
  13. 13. Rare cancers in Europe: incidence 97/100,000/year 488,000 New diagnosis/year 20% of all cancer diagnoses in a year cancers
  14. 14. INCIDENCE Distribution of : Number of cancer types Annual number of diagnoses
  15. 15. Incidence rates for rare and common cancers AGE
  16. 16. <ul><li>Variation depends on: </li></ul><ul><li>Distribution of risk factors </li></ul><ul><li>Population age </li></ul><ul><li>Case mix </li></ul><ul><li>Availability of expertise </li></ul>
  17. 17. All rare cancers by country How 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
  18. 18. All rare cancers by country How 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
  19. 19. adrenal cancers by country How many new diagnoses/year
  20. 20. Survival <ul><li>Survival corrected by competitive mortality (relative survival) </li></ul><ul><li>Net survival in clinical study </li></ul>
  21. 21. Five-year relative survival of rare and common cancers, by age 5-year survival: common cancers 64% vs rare cancers 48%
  22. 22. Five-year relative survival (%) by European country common rare
  23. 23. Rare cancers in Europe: prevalence <ul><li>4 million patients living in EU27 with a diagnosis of a rare cancer. </li></ul><ul><li>22% of the total cancer prevalence. </li></ul>
  24. 24. Rare cancers in Europe: prevalence by time since diagnosis Prevalence is relevant for health planning
  25. 25. prevalence of rare H&N as an example
  26. 26. ESO course: rare cancers chosen 40% of all rare cancers
  27. 27. 9% of all rare tumours Head and neck Crude incidence x 100,000 x year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases EPITHELIAL TUMOURS OF NASAL CAVITY AND SINUSES 0.44 2,216 47.9 2.91 14,566 Squamous cell carcinoma 0.31 1,551 49.0 2.09 10,469 Lymphoepithelial carcinoma 0.00 12 28.8 0.01 72 Undifferentiated carcinoma 0.02 88 32.5 0.13 669 Intestinal type adenocarcinoma 0.00 12 50.1 0.02 123 EPITHELIAL TUMOURS OF NASOPHARYNX 0.45 2,231 49.2 2.94 14,711 Squamous cell carcinoma 0.33 1,638 49.1 2.20 11,022 Papillary adenocarcinoma 0.00 4 58.8 0.01 29 EPITHELIAL TUMOURS OF MAJOR SALIVARY GLANDS AND SALIVARY GLAND TYPE 1.31 6,556 64.8 13.08 65,396 Epithelial tumours of major salivary glands 0.73 3,650 64.6 7.90 39,491 Salivary gland-type tumours of head and neck 0.43 2,153 69.1 4.53 22,669 EPITHELIAL TUMOURS OF OROPHARYNX 2.75 13,773 37.0 13.04 65,208 Squamous cell carcinoma 2.59 12,956 37.2 12.51 62,572 EPITHELIAL TUMOURS OF ORAL CAVITY AND LIP 4.80 23,981 59.2 34.07 170,375 Squamous cell of oral cavity 3.30 16,475 48.3 19.34 96,688 Squamous cell carcinoma of lip 1.22 6,101 91.7 12.79 63,946
  28. 28. 3.6% of all rare tumours Thyroid cancer Crude incidence x 100,000 x year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases Carcinoma of thyroid gland 3.68 18,376 88.2 61.68 308,377 Endocrine ca of thyroid gland 0.22 1,093 80.5 3.25 16,246
  29. 29. 3% of all rare tumours Rare male genital cancers Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases TUMOURS OF TESTIS AND PARATESTIS 3.15 15,679 95.96 87.8 436,638 Adenocarcinoma with variants of paratestis 0.001 7 88.96 <0.1 50 Germ cell non seminoma 1.21 6,031 94.11 24.2 166,812 Germ cell seminoma 1.71 8,518 98.47 46.8 228,916 Spermatocytic seminoma 0.03 137 92.9 9.1 3,739 Teratoma with malignant transformation 0.001 7 _ 0.4 208 ex cord tumours 0.02 109 83.04 0.4 2,189 EXTRAGONADAL GERM CELL TUMOURS 0.13 630 70.81 3.0 14,715 Extragonadal malignant immature teratomas 0.04 207 62.11 0.9 4,549 Extragonadal germ cell tumor 0.08 423 74.77 2.5 12,478
  30. 30. 0.1% of all rare tumours Adrenal cancer Crude incidence x 100,000 x year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases Carcinoma of adrenal gland 0.18 907 39.5 1.15 5,727
  31. 31. 2.6% of all rare tumours Rare female genital cancers Crude incidence x 100,000 x year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases MIXED EPITHELIAL AND MESENCHYMAL TUMOURS OF UTERUS 0.45 2,228 37.4 2.59 12,954 NON EPITHELIAL TUMOURS OF OVARY 0.43 2,166 62.5 6.69 33,457 Mixed epithelial mesenchymal tumours 0.16 783 18.2 0.49 2,474 Sex cord tumours 0.13 671 82.7 1.85 9,271 Malignant immature teratomas 0.07 338 83.2 1.50 7,519 Germ cell tumours 0.07 374 84.5 2.24 11,185 EPITHELIAL TUMOURS OF VULVA AND VAGINA 1.91 9,571 60.9 15.34 76,689 Squamous cell carcinoma 1.50 7,514 59.6 12.42 62,107 Adenocarcinoma 0.08 388 42.9 0.52 2,623 Paget's disease 0.05 255 98.0 0.47 2,350 Undifferentiated carcinoma 0.01 40 31.5 0.05 236 TROPHOBLASTIC TUMOURS OF PLACENTA 0.02 118 90.0 0.86 4,297 Choriocarcinoma of placenta 0.02 118 90.0 0.78 3,906
  32. 32. 2% of all rare tumours Rare urological cancer Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases EPITHELIAL TUMOURS OF PENIS 0.62 3,119 71.9 5.54 27,698 Squamous cell carcinoma 0.57 2,867 73.1 5.03 25,173 Adenocarcinoma 0.00 25 51.9 0.03 141 EPITHELIAL TUMOURS OF PELVIS, URETER AND URETHRA 1.59 7,947 53.5 10.96 54,793 Transitional cell carcinoma 1.37 6,868 56.0 9.86 49,281 Squamous cell carcinoma 0.05 257 32.9 0.21 1,049 Adenocarcinoma 0.04 186 47.9 0.21 1,031 Salivary gland type tumours 0.00 1 0.0 0.00 8
  33. 33. 4.5% of all rare tumours Sarcoma Incidence Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases SOFT TISSUE SARCOMA 4.75 23,755 55.8 46.86 234,289 head and neck 0.29 1,453 64.6 2.96 14,803 limbs 1.03 5,165 67.2 11.67 58,326 superficial trunk 0.47 2,358 47.5 4.07 20,349 mediastinum 0.03 134 22.7 0.11 551 heart 0.01 75 13.0 0.05 249 breast 0.19 933 78.4 2.21 11,050 uterus 0.50 2,488 50.5 4.89 24,432 Other of genitourinary tract 0.24 1,196 47.4 2.17 10,842 viscera 0.51 2,546 39.8 2.65 13,232 paratestis 0.03 163 87.5 0.31 1,528 Retroperitoneum & peritoneum 0.29 1,445 37.3 1.25 6,251 pelvis 0.01 73 34.6 0.08 409 skin 0.31 1,550 92.4 4.54 22,711 paraorbit 0.01 35 64.8 0.24 1,198 brain & other parts of CNS 0.20 999 55.2 2.19 10,971 Embryonal rhabdomyosarcoma 0.06 305 67.3 1.67 8,373 Alveolar rhabdomyosarcama 0.03 162 41.3 0.20 989 Ewing’s family tumours 0.02 119 52.1 0.26 1,311 GIST 0.07 330 70.5 0.44 2,193
  34. 34. 5.2% of all rare tumours Rare CNS cancers Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases GLIAL TUMOURS OF CENTRAL NERVOUS SYSTEM (CNS) AND PINEAL GLAND 5.35 26,726 20.0 26.29 131,433 Astrocytic tumours of CNS 4.79 23,963 15.1 20.42 102,113 Oligodendroglial tumours of CNS 0.35 1,765 54.0 2.65 13,254 Ependymal tumours of CNS 0.20 997 71.3 3.84 19,223 NON GLIAL TUMOURS OF CENTRAL NERVOUS SYSTEM (CNS) AND PINEAL GLAND 0.22 1,120 53.1 4.74 23,689 Embryonal tumours of CNS 0.22 1,089 53.2 4.32 21,580 Choroid plexus carcinoma of CNS 0.01 31 46.9 0.35 1,744
  35. 35. 2.3% of all rare tumours Neuroendocrine Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases NEUROENDOCRINE TUMOURS 2.52 12,619 50.8 20.10 100,515 Well differentiated endocrine tumours, carcinoid 0.37 1,826 32.2 1.57 7,831 Well differentiated endocrine tumours, atypical carcinoid 0.00 4 101.8 0.01 35 Poorly differentiated endocrine carcinoma (lung small cell carcinoma excluded) 0.52 2,605 12.9 1.34 6,713 Mixed endocrine-exocrine carcinoma 0.00 10 34.8 0.02 97 Endocrine carcinoma of thyroid gland 0.22 1,093 80.5 3.25 16,246 Well differentiated non functioning endocrine carcinoma of pancreas and of digestive tract 1.25 6,260 64.4 12.80 64,017 Well differentiated functioning endocrine carcinoma of pancreas and of digestive tract 0.02 123 51.3 0.22 1,075 Endocrine carcinoma of skin 0.13 667 57.6 0.86 4,295
  36. 36. 5.8% of all rare tumours Rare digestive cancers Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases EPITHELIAL TUMOURS OF SMALL INTESTINE 0.73 3,627 25.4 2.67 13,344 Adenocarcinoma 0.57 2,846 25.7 2.21 11,040 Squamous cell carcinoma 0.01 31 20.7 0.03 126 EPITHELIAL TUMOURS OF ANAL CANAL 1.10 5,501 55.5 8.16 40,796 Squamous cell carcinoma 0.61 3,072 61.5 5.88 29,416 Adenocarcinoma 0.26 1,294 42.4 1.07 5,360 Basaloid carcinoma 0.12 612 61.1 0.95 4,774 Paget's disease 0.00 20 59.9 0.02 121 EPITHELIAL TUMOURS OF GALLBLADDER AND EXTRAHEPATIC BILIARY DUCT (EBT) 4.42 22,099 12.7 6.83 34,148 Adenocarcinoma 2.64 13,197 15.1 5.37 26,838 Combined hepatocellular carcinoma and cholangiocarcinoma 0.00 9 39.2 0.01 30 Squamous cell carcinoma 0.04 181 12.2 0.05 228
  37. 37. 2.5% of all rare tumours Rare thoracic cancers Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases EPITHELIAL TUMOURS OF TRACHEA 0.13 672 12.2 0.28 1,403 Squamous cell carcinoma 0.08 409 8.5 0.12 605 Adenocarcinoma 0.01 67 7.6 0.02 120 Salivary gland type tumours 0.01 49 56.2 0.11 526 EPITHELIAL TUMOURS OF THYMUS 0.17 841 57.4 1.40 6,998 Malignant thymoma 0.14 690 60.6 1.22 6,086 Squamous cell carcinoma 0.00 23 44.6 0.02 119 Undifferentiated carcinoma 0.00 12 18.2 0.00 16 Lymphoepithelial carcinoma 0.00 4 67.6 0.01 61 Adenocarcinoma 0.00 10 32.8 0.01 40 MALIGNANT MESOTHELIOMA 1.91 9,530 5.5 2.38 11,902 Mesothelioma of pleura and pericardium 1.61 8,050 4.9 1.97 9,874 Mesothelioma of peritoneum and tunica vaginalis 0.13 625 11.3 0.22 1,077
  38. 38. 0.2% of all rare tumours CNS tumours (2) Crude incidence per 100,000 per year Expected new cases per year Relative 5-year survival (%) Complete prevalence per 100,000 Prevalent Cases MALIGNANT MENINGIOMAS 0.15 755 61.8 1.75 8,743 GLIAL TUMOURS OF CRANIAL AND PERIPHERAL NERVES, AUTONOMIC NERVOUS SYSTEM AND PARAGANGLIA 0.01 51 86.5 0.41 2,040 Astrocytic tumours of cranial and peripheral nerves, autonomic nervous system and paraganglia 0.00 25 68.7 0.16 824 Ependymal tumours of cranial and peripheral nerves and autonomic nervous system 0.01 26 104.4 0.10 475 NON-GLIAL TUMOURS OF CRANIAL AND PERIPHERAL NERVES, AUTONOMIC NERVOUS SYSTEM AND PARAGANGLIA 0.10 493 63.5 1.19 5,926 Embryonal tumours of cranial and peripheral nerves, autonomic nervous system and paraganglia 0.07 369 67.0 0.88 4,388 Paraganglioma 0.02 124 51.0 0.27 1,352
  39. 39. What I am expect with this course <ul><li>Transferring know-how between clinicians, epidemiolgists, patologists , advocacy groups. </li></ul><ul><li>ideas for collaborative projects </li></ul><ul><li>creation of networks (for rare cancers treatment, research, European rare cancer information network, …) </li></ul><ul><li>improve the organization of rare cancers treatment </li></ul>
  40. 40. <ul><li>RARECARE: http://www.rarecare.eu/ </li></ul>Enjoy the meeting!
  41. 41. Conclusions: rare cancers burden <ul><li>Incidence = 488,000 New diagnosis/year </li></ul><ul><ul><ul><ul><ul><li>20% of all cancer diagnoses/year </li></ul></ul></ul></ul></ul><ul><li>Prevalence = 4 million patients </li></ul><ul><ul><ul><ul><ul><li> 22% of the total cancer prevalence </li></ul></ul></ul></ul></ul><ul><li>Survival = common cancers 64% vs rare cancers 48% </li></ul>

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