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. 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
26. ESO course: rare cancers chosen 40% of all rare cancers
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. 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. 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. 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. 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. 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
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. 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. 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. 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. 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