RARE EPITHELIAL TUMOURS OF THE 16 % OF DIGESTIVE SYSTEM TUMOURS ARE RARE DIGESTIVE SYSTEM EPITHELIAL TUMOURS 2 262 EPITHELIAL TUMOURS OF OESOPHAGUS 81 % OF RARE EPITHELIAL TUMOURS 271 RARE EPITHELIAL TUMOURS 1 OUT OF ALL TUMOURS OF STOMACH IN EACH SITE 696 EPITHELIAL TUMOURS OF SMALL INTESTINE 56 87 RARE EPITHELIAL TUMOURS <1 INCIDENCE OF COLON 17 532 97 RARE EPITHELIAL TUMOURS OF RECTUM 1 ESTIMATED NEW CASES 1 143 EPITHELIAL TUMOURS OF ANAL CANAL 97 ITALY, 2015 RARE EPITHELIAL TUMOURS 71 OF PANCREAS 1 EPITHELIAL TUMOURS OF LIVER 7 291 AND INTRAHEPATIC BILE TRACT 53 EPITHELIAL TUMOURS OF GALLBLADDER 5 483 AND EXTRAHEPATIC BILIARY TRACT 99 132 MESOTHELIOMA OF PERITONEUM PREVALENCE SURVIVAL 43 452 100% ESTIMATED PREVALENT CASES ITALY, 2010 50% 20% 0 1 5 YEARS AFTER DIAGNOSIS SOURCE: AIRTUM. ITALIAN CANCER FIGURES–REPORT 2015 RARE EPITHELIAL TUMOURS OF THE DIGESTIVE SYSTEM I tumori in Italia • Rapporto AIRTUM 2015 INCIDENCE RARE EPITHELIAL TUMOURS OF THE DIGESTIVE SYSTEM. Crude incidence (rate per 100,000/year) and 95% confidence interval (95% CI), observed cases and proportion of rare cancers on all (common + rare) cancers by site. Rates with 95% CI by sex and age. Estimated new cases at 2015 in Italy. AIRTUM POOL (period of diagnosis 2000-2010) ITALY SEX AGE MALE FEMALE 0-54 yrs 55-64 yrs 65+ yrs ESTIMATED NEW CASES RATE 95% CI RATE 95% CI RATE 95% CI RATE 95% CI RATE 95% CI RATE 95% CI 2015 OBSERVED CASES OBSERVED (No.) EPITHELIAL RARE SITE BY CANCERS (%) RARE EPITHELIAL TUMOURS 26.11 25.89-26.32 57 891 16% 32.11 31.78-32.45 20.48 20.22-20.74 3.74 3.64-3.83 40.15 39.40-40.91 93.99 93.08-94.90 17 532 OF THE DIGESTIVE SYSTEM EPITHELIAL TUMOURS OF OESOPHAGUS 3.38 3.30-3.45 7 488 81% 5.35 5.21-5.49 1.53 1.46-1.60 0.63 0.59-0.67 6.90 6.60-7.22 10.59 10.29-10.90 2 262 Squamous cell carcinoma with variants of oesophagus 2.44 2.37-2.50 5 405 3.74 3.63-3.86 1.21 1.15-1.28 0.47 0.43-0.50 5.43 5.15-5.71 7.33 7.07-7.58 1 619 Adenocarcinoma with variants of oesophagus 0.90 0.86-0.94 1 993 1.54 1.47-1.62 0.30 0.26-0.33 0.15 0.13-0.17 1.41 1.27-1.55 3.14 2.97-3.31 616 Salivary gland type tumours of oesophagus <0.01 0.00-0.00 4 NE – NE – NE – NE – NE –2 Undifferentiated carcinoma of oesophagus 0.04 0.03-0.05 86 0.06 0.04-0.07 0.02 0.01-0.03 <0.01 0.00-0.01 0.07 0.04-0.11 0.13 0.10-0.17 27 RARE EPITHELIAL TUMOURS OF STOMACH 0.40 0.37-0.42 879 1% 0.50 0.46-0.54 0.30 0.27-0.33 0.06 0.05-0.07 0.62 0.53-0.72 1.42 1.31-1.53 271 Squamous cell carcinoma with variants of stomach 0.09 0.08-0.11 204 0.14 0.12-0.17 0.04 0.03-0.06 0.01 0.01-0.02 0.18 0.14-0.24 0.31 0.26-0.37 62 Salivary gland type tumours of stomach <0.01 0.00-0.00 2 NE – NE – NE – NE – NE –1 Undifferentiated carcinoma of stomach 0.30 0.28-0.33 673 0.35 0.32-0.39 0.26 0.23-0.29 0.05 0.04-0.06 0.43 0.36-0.51 1.11 1.01-1.21 208 EPITHELIAL TUMOURS OF SMALL INTESTINE 1.02 0.98-1.06 2 261 56% 1.15 1.09-1.22 0.90 0.84-0.95 0.20 0.18-0.22 1.40 1.26-1.55 3.60 3.42-3.78 696 Adenocarcinoma with variants of small intestine 0.78 0.74-0.81 1 722 0.91 0.85-0.97 0.65 0.61-0.70 0.16 0.14-0.19 1.19 1.06-1.32 2.62 2.47-2.77 521 Squamous cell carcinoma with variants of small intestine <0.01 0.01-0.01 21 0.01 0.01-0.02 <0.01 0.00-0.01 <0.01 0.00-0.00 <0.01 0.00-0.03 0.04 0.02-0.06 6 RARE EPITHELIAL TUMOURS OF COLON 0.13 0.12-0.15 293 0.2% 0.11 0.09-0.13 0.15 0.13-0.17 0.05 0.04-0.06 0.18 0.14-0.24 0.38 0.32-0.44 87 Squamous cell carcinoma with variants of colon 0.03 0.03-0.04 74 0.02 0.01-0.03 0.04 0.03-0.06 0.01 0.01-0.02 0.06 0.03-0.09 0.10 0.07-0.13 23 Fibromixoma and low grade 0.10 0.09-0.11 219 0.09 0.07-0.11 0.11 0.09-0.13 0.04 0.03-0.05 0.13 0.09-0.18 0.28 0.23-0.33 65 mucinous adenocarcinoma of the appendix RARE EPITHELIAL TUMOURS OF RECTUM 0.14 0.13-0.16 318 1% 0.08 0.06-0.10 0.20 0.18-0.23 0.05 0.04-0.06 0.20 0.15-0.26 0.43 0.37-0.50 97 Squamous cell carcinoma with variants of rectum 0.14 0.13-0.16 318 0.08 0.06-0.10 0.20 0.18-0.23 0.05 0.04-0.06 0.20 0.15-0.26 0.43 0.37-0.50 97 EPITHELIAL TUMOURS OF ANAL CANAL 1.69 1.64-1.75 3 750 97% 1.40 1.33-1.47 1.97 1.89-2.05 0.47 0.43-0.50 2.53 2.35-2.73 5.36 5.15-5.58 1 143 Squamous cell carcinoma with variants of anal canal 0.92 0.88-0.96 2 042 0.56 0.52-0.61 1.26 1.19-1.32 0.34 0.31-0.37 1.52 1.38-1.68 2.52 2.38-2.68 611 Adenocarcinoma with variants of anal canal 0.60 0.57-0.64 1 338 0.71 0.66-0.77 0.50 0.46-0.54 0.10 0.08-0.12 0.85 0.74-0.96 2.18 2.04-2.32 411 Paget’s disease of anal canal <0.01 0.00-0.01 8 NE – NE – NE – NE – NE –2 RARE EPITHELIAL TUMOURS OF PANCREAS 0.11 0.10-0.12 241 1% 0.12 0.10-0.14 0.10 0.08-0.12 0.04 0.03-0.05 0.17 0.12-0.22 0.32 0.27-0.38 71 Squamous cell carcinoma with variants of pancreas 0.02 0.01-0.02 39 0.02 0.01-0.03 0.01 0.01-0.02 <0.01 0.00-0.01 0.03 0.01-0.05 0.05 0.03-0.08 12 Acinar cell carcinoma of pancreas 0.04 0.03-0.05 94 0.06 0.05-0.08 0.02 0.02-0.04 0.01 0.01-0.02 0.08 0.05-0.13 0.12 0.09-0.16 28 Mucinous cystadenocarcinoma of pancreas 0.03 0.02-0.03 56 0.02 0.01-0.03 0.03 0.02-0.04 <0.01 0.00-0.01 0.03 0.01-0.06 0.08 0.06-0.11 16 Intraductal papillary mucinous carcinoma 0.02 0.01-0.02 34 0.02 0.01-0.03 0.01 0.01-0.02 <0.01 0.00-0.01 0.03 0.01-0.05 0.05 0.03-0.08 10 invasive of pancreas Solid pseudopapillary carcinoma of pancreas <0.01 0.00-0.01 13 NE – NE – NE – NE – NE –4 Serous cystadenocarcinoma of pancreas <0.01 0.00-0.00 3 NE – NE – NE – NE – NE –1 Carcinoma with osteoclast-like giant cells of pancreas <0.01 0.00-0.00 2 NE – NE – NE – NE – NE –1 EPITHELIAL TUMOURS OF LIVER 11.05 10.91-11.19 24 497 53% 16.32 16.08-16.57 6.10 5.96-6.25 1.53 1.47-1.59 18.62 18.11-19.14 38.93 38.35-39.52 7 291 AND INTRAHEPATIC BILE TRACT Hepatocellular carcinoma of liver and IBT 9.37 9.25-9.50 20 784 14.22 14.00-14.45 4.83 4.70-4.96 1.22 1.17-1.28 15.63 15.17-16.11 33.39 32.85-33.94 6 195 Hepatocellular carcinoma, fibrolamellar of liver and IBT 0.09 0.07-0.10 189 0.13 0.11-0.16 0.04 0.03-0.06 0.01 0.01-0.02 0.13 0.09-0.18 0.30 0.26-0.36 55 Cholangiocarcinoma of IBT 0.90 0.86-0.94 2 003 1.09 1.03-1.16 0.73 0.68-0.78 0.18 0.16-0.20 1.65 1.50-1.81 2.91 2.76-3.08 593 Adenocarcinoma with variants of liver and IBT 0.65 0.61-0.68 1 432 0.83 0.77-0.88 0.48 0.44-0.52 0.11 0.09-0.13 1.14 1.02-1.28 2.17 2.04-2.32 422 Undifferentiated carcinoma of liver and IBT 0.02 0.01-0.02 40 0.02 0.02-0.03 0.01 0.01-0.02 <0.01 0.00-0.01 0.04 0.02-0.07 0.06 0.04-0.09 12 Squamous cell carcinoma with variants of liver and IBT 0.02 0.01-0.02 36 0.02 0.01-0.03 0.01 0.01-0.02 <0.01 0.00-0.00 0.02 0.01-0.04 0.07 0.04-0.09 11 Bile duct cystadenocarcinoma of IBT <0.01 0.00-0.01 13 NE – NE – NE – NE – NE –4 EPITHELIAL TUMOURS OF GALLBLADDER 7.99 7.87-8.11 17 715 99% 6.84 6.68-7.00 9.07 8.89-9.24 0.66 0.62-0.70 9.15 8.79-9.52 32.37 31.84-32.91 5 483 AND EXTRAHEPATIC BILIARY TRACT Adenocarcinoma with variants of gallbladder 2.03 1.97-2.09 4 498 1.31 1.24-1.38 2.71 2.61-2.80 0.20 0.18-0.23 3.14 2.93-3.36 7.59 7.34-7.85 1 328 Adenocarcinoma with variants of EBT 2.24 2.17-2.30 4 960 2.54 2.44-2.64 1.95 1.87-2.04 0.31 0.28-0.34 3.67 3.44-3.90 7.94 7.68-8.21 1 479 Squamous cell carcinoma of gallbladder and EBT 0.04 0.03-0.05 84 0.02 0.02-0.04 0.05 0.04-0.07 <0.01 0.00-0.01 0.06 0.04-0.10 0.14 0.11-0.18 25 MESOTHELIOMA OF PERITONEUM 0.20 0.18-0.22 449 NA 0.25 0.22-0.28 0.16 0.14-0.18 0.06 0.05-0.07 0.38 0.31-0.46 0.59 0.52-0.66 132 NE: not estimable because 15 or less incident cases were observed IBT: intrahepatic bile tract EBT: extrahepatic bile tract NA: not applicable Epidemiol Prev 40 (1) Suppl 2:1-120 44 ® Ulteriori dati disponibili sul sito: www.registri-tumori.it RARE EPITHELIAL TUMOURS OF THE DIGESTIVE SYSTEM I tumori in Italia • Rapporto AIRTUM 2015 SURVIVAL RARE EPITHELIAL TUMOURS OF THE DIGESTIVE SYSTEM.