ITALIAN CANCER FIGURES - REPORT 2015: The burden of rare cancers in Italy = I TUMORI IN ITALIA - RAPPORTO 2015: I tumori rari in Italia

OBJECTIVES: This collaborative study, based on data collected by the network of Italian Cancer Registries (AIRTUM), describes the burden of rare cancers in Italy. Estimated number of new rare cancer cases yearly diagnosed (incidence), proportion of patients alive after diagnosis (survival), and estimated number of people still alive after a new cancer diagnosis (prevalence) are provided for about 200 different cancer entities. MATERIALS AND METHODS: Data herein presented were provided by AIRTUM population- based cancer registries (CRs), covering nowadays 52% of the Italian population. This monograph uses the AIRTUM database (January 2015), which includes all malignant cancer cases diagnosed between 1976 and 2010. All cases are coded according to the International Classification of Diseases for Oncology (ICD-O-3). Data underwent standard quality checks (described in the AIRTUM data management protocol) and were checked against rare-cancer specific quality indicators proposed and published by RARECARE and HAEMACARE (www.rarecarenet.eu; www.haemacare.eu). The definition and list of rare cancers proposed by the RARECAREnet "Information Network on Rare Cancers" project were adopted: rare cancers are entities (defined as a combination of topographical and morphological codes of the ICD-O-3) having an incidence rate of less than 6 per 100,000 per year in the European population. This monograph presents 198 rare cancers grouped in 14 major groups. Crude incidence rates were estimated as the number of all new cancers occurring in 2000-2010 divided by the overall population at risk, for males and females (also for gender-specific tumours).The proportion of rare cancers out of the total cancers (rare and common) by site was also calculated. Incidence rates by sex and age are reported. The expected number of new cases in 2015 in Italy was estimated assuming the incidence in Italy to be the same as in the AIRTUM area. One- and 5-year relative survival estimates of cases aged 0-99 years diagnosed between 2000 and 2008 in the AIRTUM database, and followed up to 31 December 2009, were calculated using complete cohort survival analysis. To estimate the observed prevalence in Italy, incidence and follow-up data from 11 CRs for the period 1992-2006 were used, with a prevalence index date of 1 January 2007. Observed prevalence in the general population was disentangled by time prior to the reference date (≤2 years, 2-5 years, ≤15 years). To calculate the complete prevalence proportion at 1 January 2007 in Italy, the 15-year observed prevalence was corrected by the completeness index, in order to account for those cancer survivors diagnosed before the cancer registry activity started. The completeness index by cancer and age was obtained by means of statistical regression models, using incidence and survival data available in the European RARECAREnet data. RESULTS: In total, 339,403 tumours were included in the incidence analysis. The annual incidence rate (IR) of all 198 rare cancers in the period 2000-2010 was 147 per 100,000 per year, corresponding to about 89,000 new diagnoses in Italy each year, accounting for 25% of all cancer. Five cancers, rare at European level, were not rare in Italy because their IR was higher than 6 per 100,000; these tumours were: diffuse large B-cell lymphoma and squamous cell carcinoma of larynx (whose IRs in Italy were 7 per 100,000), multiple myeloma (IR: 8 per 100,000), hepatocellular carcinoma (IR: 9 per 100,000) and carcinoma of thyroid gland (IR: 14 per 100,000). Among the remaining 193 rare cancers, more than two thirds (No. 139) had an annual IR <0.5 per 100,000, accounting for about 7,100 new cancers cases; for 25 cancer types, the IR ranged between 0.5 and 1 per 100,000, accounting for about 10,000 new diagnoses; while for 29 cancer types the IR was between 1 and 6 per 100,000, accounting for about 41,000 new cancer cases. Among all rare cancers diagnosed in Italy, 7% were rare haematological diseases (IR: 41 per 100,000), 18% were solid rare cancers. Among the latter, the rare epithelial tumours of the digestive system were the most common (23%, IR: 26 per 100,000), followed by epithelial tumours of head and neck (17%, IR: 19) and rare cancers of the female genital system (17%, IR: 17), endocrine tumours (13% including thyroid carcinomas and less than 1% with an IR of 0.4 excluding thyroid carcinomas), sarcomas (8%, IR: 9 per 100,000), central nervous system tumours and rare epithelial tumours of the thoracic cavity (5%with an IR equal to 6 and 5 per 100,000, respectively). The remaining (rare male genital tumours, IR: 4 per 100,000; tumours of eye, IR: 0.7 per 100,000; neuroendocrine tumours, IR: 4 per 100,000; embryonal tumours, IR: 0.4 per 100,000; rare skin tumours and malignant melanoma of mucosae, IR: 0.8 per 100,000) each constituted <4% of all solid rare cancers. Patients with rare cancers were on average younger than those with common cancers. Essentially, all childhood cancers were rare, while after age 40 years, the common cancers (breast, prostate, colon, rectum, and lung) became increasingly more frequent. For 254,821 rare cancers diagnosed in 2000-2008, 5-year RS was on average 55%, lower than the corresponding figures for patients with common cancers (68%). RS was lower for rare cancers than for common cancers at 1 year and continued to diverge up to 3 years, while the gap remained constant from 3 to 5 years after diagnosis. For rare and common cancers, survival decreased with increasing age. Five-year RS was similar and high for both rare and common cancers up to 54 years; it decreased with age, especially after 54 years, with the elderly (75+ years) having a 37% and 20% lower survival than those aged 55-64 years for rare and common cancers, respectively. We estimated that about 900,000 people were alive in Italy with a previous diagnosis of a rare cancer in 2010 (prevalence). The highest prevalence was observed for rare haematological diseases (278 per 100,000) and rare tumours of the female genital system (265 per 100,000). Very low prevalence (<10 prt 100,000) was observed for rare epithelial skin cancers, for rare epithelial tumours of the digestive system and rare epithelial tumours of the thoracic cavity. COMMENTS: One in four cancers cases diagnosed in Italy is a rare cancer, in agreement with estimates of 24% calculated in Europe overall. In Italy, the group of all rare cancers combined, include 5 cancer types with an IR>6 per 100,000 in Italy, in particular thyroid cancer (IR: 14 per 100,000).The exclusion of thyroid carcinoma from rare cancers reduces the proportion of them in Italy in 2010 to 22%. Differences in incidence across population can be due to the different distribution of risk factors (whether environmental, lifestyle, occupational, or genetic), heterogeneous diagnostic intensity activity, as well as different diagnostic capacity; moreover heterogeneity in accuracy of registration may determine some minor differences in the account of rare cancers. Rare cancers had worse prognosis than common cancers at 1, 3, and 5 years from diagnosis. Differences between rare and common cancers were small 1 year after diagnosis, but survival for rare cancers declined more markedly thereafter, consistent with the idea that treatments for rare cancers are less effective than those for common cancers. However, differences in stage at diagnosis could not be excluded, as 1- and 3-year RS for rare cancers was lower than the corresponding figures for common cancers. Moreover, rare cancers include many cancer entities with a bad prognosis (5-year RS <50%): cancer of head and neck, oesophagus, small intestine, ovary, brain, biliary tract, liver, pleura, multiple myeloma, acute myeloid and lymphatic leukaemia; in contrast, most common cancer cases are breast, prostate, and colorectal cancers, which have a good prognosis. The high prevalence observed for rare haematological diseases and rare tumours of the female genital system is due to their high incidence (the majority of haematological diseases are rare and gynaecological cancers added up to fairly high incidence rates) and relatively good prognosis. The low prevalence of rare epithelial tumours of the digestive system was due to the low survival rates of the majority of tumours included in this group (oesophagus, stomach, small intestine, pancreas, and liver), regardless of the high incidence rate of rare epithelial cancers of these sites. This AIRTUM study confirms that rare cancers are a major public health problem in Italy and provides quantitative estimations, for the first time in Italy, to a problem long known to exist. This monograph provides detailed epidemiologic indicators for almost 200 rare cancers, the majority of which (72%) are very rare (IR<0.5 per 100,000). These data are of major interest for different stakeholders. Health care planners can find useful information herein to properly plan and think of how to reorganise health care services. Researchers now have numbers to design clinical trials considering alternative study designs and statistical approaches. Population-based cancer registries with good quality data are the best source of information to describe the rare cancer burden in a population

I tumori dei bambini e degli adolescenti nella provincia di Palermo: i dati del Registro Tumori di Palermo e provincia.

Tumori infantili; Registri Tumori; Incidenz

Comparison of paediatric cancers outcomes between Palermo Province (Sicily) and Southern Europe

Background: Italy has one of the highest paediatric cancer incidence rate in Europe. We compared cancer incidence and survival in children (0-14 years) and adolescents (15-19 years) resident in Palermo Province with statistics derived from Italian and European surveillance systems. Methods: We included all incident cancer cases, malignant tumours and non-malignant neoplasm of central nervous system (benign and uncertain whether malignant or benign), detected in children and adolescents by the Palermo Province Cancer Registry between 2003 and 2012. A joinpoint regression model was applied. Annual Average Percentage Changes were calculated. The Besag–York–Mollie model was used to detect any cluster. The 5-year survival analysis was computed using Kaplan-Meier and actuarial methods. Results: We identified 555 paediatric cancer incident cases (90% ‘‘malignant tumours only’’). No difference in incidence rates was highlight between Palermo Province and Italy 26 registries and between RTPP and Southern Europe. No joinpoint or significant trend was identified and no cluster was detected. The 5-year overall survival didn’t differ between Palermo Province and Italian AIRTUM pool. A borderline higher statistically significant survival was observed in age-group 1-4 when comparing PPCR to EUROCARE-5. Conclusions: The epidemiological surveillance documented in the Palermo Province a paediatric cancer burden in line with southern Europe and a 5-year survival in line with EUROCARE-5, together with a borderline higher survival in age-group 1-4. The study supports the supplementary role of general population-based cancer registries to provide paediatric cancer surveillance of local communities. The adoption of the new guidelines and recommendations, on which staging systems should be adopted by population-based cancer registries for the major childhood cancers, will make it easy to perform comparative studies on incidence and other outcomes of interest, particularly survival. Key messages: Use of population-based cancer registries to assess and control the impact of cancer diseases. Supplementary role of population-based cancer registries to carry out infant cancer surveillance

I TUMORI INFANTILI E DEGLI ADOLESCENTI: TREND DI INCIDENZA E STUDIO DI EVENTUALI CLUSTER. CONFRONTO TRA LE PROVINCE DI PALERMO E DI CALTANISSETTA NEL PERIODO 2007-2012

INTRODUZIONE: Nonostante i progressi nelle strategie terapeutiche e il conseguente miglioramento della sopravvivenza, i tumori infantili rappresentano un importante problema di salute pubblica. \uc8 stato effettuato un confronto tra l\u2019incidenza dei tumori infantili (0-14 anni) e degli adolescenti (15-19 anni) nelle province di Palermo e di Caltanissetta, nel periodo 2007-2012. MATERIALI E METODI: Sono stati analizzati 453 nuovi casi di tumore maligno e i non maligni del SNC in soggetti <20 anni, registrati dal Registro Tumori di Palermo Provincia (RTPP) e dal Registro Tumori di Ragusa e Caltanissetta (RTRC) tra il 2007 e il 2012. Variazioni delle medie annue percentuali sono state calcolate per i due pool ed eventuali variazioni nell\u2019andamento temporale dei tassi di incidenza standardizzati (SIR) sulla popolazione europea sono state esplorate applicando un modello di regressione joinpoint. Il modello Besag-York-Molly e l\u2019approccio GAM (Geographical Analysis Machine) sono stati usati per rilevare eventuali cluster. Le analisi sono state realizzate coi software SEERstat ver 4.1.1 (2014) e RStudio ver 0.98.945 e con R ver 3.1.0 (2014). RISULTATI: I tassi di incidenza et\ue0 speci ci della provincia di Caltanissetta sono risultati pi\uf9 elevati di quelli della provincia di Palermo, ad eccezione della classe 1-4 anni. Un trend in aumento statisticamente signi cativo \ue8 stato documentato nella provincia di Palermo per i SIR nelle femmine della classe di et\ue0 0-14 e nella provincia di Caltanissetta per i tassi et\ue0 speci ci della classe di et\ue0 15-19, sia nell\u2019intera popolazione (M + F) che nelle femmine. Un potenziale cluster (SIR= 3; p-vale=0,39) \ue8 stato rilevato nella classe di et\ue0 15-19 in un\u2019area della provincia di Caltanissetta in contiguit\ue0 con le Madonie. CONCLUSIONI: I tassi di incidenza di tumore maligno e non maligno del SNC nel periodo 2007-2012 sono risultati pi\uf9 elevati nella provincia di Caltanissetta rispetto alla provincia di Palermo. Sebbene si sia registrato un trend in aumento per alcuni gruppi in entrambe le provincie, l\u2019assenza di joinpoint suggerisce come nel periodo di studio non si siano veri cati eventi di rilievo epidemiologico. Si ritiene opportuno approfondirne lo studio del potenziale cluster rilevato, superando i limiti dei con ni amministrativi di riferimento per i due registri