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The incidence of early-onset cancers in adolescents and young adults (AYA) has been increasing worldwide since the 1990s. In Italy, a significant increased rate of 1.6 % per year has been reported for early-onset cancers among females between 2008 and 2016. This is mainly attributable to melanoma, thyroid, breast and endometrial cancer. The aim of our work was to describe temporal trends of the main established lifestyle risk factors (tobacco use, alcohol consumption, obesity, physical inactivity, dietary westernization and reproductive factors) over the last 20 years in the Italian AYA population. Available data on behavioural risk factors, individual and household daily life have been obtained and elaborated from PASSI, ISTAT and Eurostat reports. Lowering age of smoking initiation, an increase in alcohol drinkers among young females, and an obesity and overweight epidemic, particularly among children and adolescents as a result of physical inactivity and dietary habits, may be contributing factors behind this cancer epidemic, especially among females. In-depth investigations are needed to understand the exact role of each contributing factor, the effects of exposure to nicotine-containing products and environmental factors such as endocrine disruptors that could play a role in this phenomenon., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare that are relevant to the content of this manuscript., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: We used the comprehensive definition of AYA (age 15 to 39 years) to update 5-year relative survival (RS) estimates for AYAs in Europe and across countries and to evaluate improvements in survival over time., Methods: We used data from EUROCARE-6. We analysed 700,000 AYAs with cancer diagnosed in 2000-2013 (follow-up to 2014). We focused the analyses on the 12 most common cancers in AYA. We used period analysis to estimate 5-year RS in Europe and 5-year RS differences in 29 countries (2010-2014 period estimate) and over time (2004-06 vs. 2010-14 period estimates)., Findings: 5-year RS for all AYA tumours was 84%, ranging from 70% to 90% for most of the 12 tumours analysed. The exceptions were acute lymphoblastic leukaemia, acute myeloid leukaemia, and central nervous system tumours, presenting survival of 59%, 61%, and 62%, respectively. Differences in survival were observed among European countries for all cancers, except thyroid cancers and ovarian germ-cell tumours. Survival improved over time for most cancers in the 15- to 39-year-old age group, but for fewer cancers in adolescents and 20- to 29-year-olds., Interpretation: This is the most comprehensive study to report the survival of 12 cancers in AYAs in 29 European countries. We showed variability in survival among countries most likely due to differences in stage at diagnosis, access to treatment, and lack of referral to expert centres. Survival has improved especially for haematological cancers. Further efforts are needed to improve survival for other cancers as well, especially in adolescents., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: A strong relationship has been observed between comorbidities and the risk of severe/fatal COVID-19 manifestations, but no score is available to evaluate their association in cancer patients. To make up for this lacuna, we aimed to develop a comorbidity score for cancer patients, based on the Lombardy Region healthcare databases., Methods: We used hospital discharge records to identify patients with a new diagnosis of solid cancer between February and December 2019; 61 comorbidities were retrieved within 2 years before cancer diagnosis. This cohort was split into training and validation sets. In the training set, we used a LASSO-logistic model to identify comorbidities associated with the risk of developing a severe/fatal form of COVID-19 during the first pandemic wave (March-May 2020). We used a logistic model to estimate comorbidity score weights and then we divided the score into five classes (<=-1, 0, 1, 2-4, >=5). In the validation set, we assessed score performance by areas under the receiver operating characteristic curve (AUC) and calibration plots. We repeated the process on second pandemic wave (October-December 2020) data., Results: We identified 55,425 patients with an incident solid cancer. We selected 21 comorbidities as independent predictors. The first four score classes showed similar probability of experiencing the outcome (0.2% to 0.5%), while the last showed a probability equal to 5.8%. The score performed well in both the first and second pandemic waves: AUC 0.85 and 0.82, respectively. Our results were robust for major cancer sites too (i.e., colorectal, lung, female breast, and prostate)., Conclusions: We developed a high performance comorbidity score for cancer patients and COVID-19. Being based on administrative databases, this score will be useful for adjusting for comorbidity confounding in epidemiological studies on COVID-19 and cancer impact., (© 2022 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Background: Data on late mortality from pediatric germ cell tumors (GCTs) are limited to small case series. Our population-based study aimed to investigate excess risk of death in survivors of GCT in childhood and adolescence, whether long-term mortality changed over time and by period of diagnosis., Methods: The PanCare Childhood and Adolescent Cancer Survivor Care and Follow-Up Studies (PanCareSurFup) cohort includes 2773 five-year survivors diagnosed under 21 years of age with gonadal and extragonadal GCT (from 1940 to 2008). We calculated standardized mortality ratios (SMRs) and absolute excess risks (AERs). We fitted a Cox's model to assess the impact of treatment period. We estimated 10-year survival and calculated average percentage changes between periods of diagnosis (1970-1979, 1980-1989, 1990-1999) to assess whether late mortality decreased., Results: GCT survivors had an almost four-fold excess risk of dying compared to general population. The risk of death for patients treated after 1980 was nearly halved compared to patients treated before 1980. Survivors diagnosed in 1990-1999 had a 10-year survival rate of 99%, which was 2.4% and 1.1% higher than for patients treated in 1970-1979 and 1980-1989, respectively., Conclusions: This is the largest population-based study in Europe and showed a decrease in long-term mortality for survivors of GCTs in childhood and adolescence over the last decades. After the introduction of platinum compound in 1980, which is a paradigm of success compared to the previous treatments, no major changes in drug therapies have been made to treat GCTs in the last 40 years. However, GCT survivors maintain an excessive risk of death that requires long-term care., (© 2022 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Background: As a system of European Reference Networks (ERNs) emerges, the differences in quality of care for patients with rare cancers may increase at national level. We aimed to elucidate the processes and healthcare planning principles through which the reference centres (RCs) for rare cancers are embedded in national health systems., Methods: We used a multiple case-study design based on the experiences of Czechia, Finland, France, Italy, Lithuania and Spain. Using sarcoma as an example of rare cancer, 52 semi-structured interviews were conducted during on-site visits, including a multidisciplinary group of professionals, Ministry of Health professionals, patient representatives and European policymakers., Results: The comparative analysis showed substantial heterogeneity in the processes for formalizing RCs' status and in their levels of integration in the different health systems, but two models (centre-based and the network-based) can be envisaged at national level. RCs for rare cancers were legally established only in France and Spain. Expert clinicians cooperate in a structured way, using network mechanisms, in France and Italy, and these countries, plus Finland and Lithuania, had a referral system to facilitate patients' access from non-expert centres to RCs. Seven key healthcare planning principles in instituting RCs at the national level were identified., Conclusions: The conditions governing patient access to treatment centres-whether RCs or not-are decided at the national level. It is advisable to progressively align the European and national levels so that the RCs that participate in the ERNs also play a significant role at the national level., (© The Author(s) 2022. Published by Oxford University Press on behalf of the European Public Health Association.)

Our aim was to analyse, on a population level, the year-long decline in cancer diagnoses in the region of Lombardy (Italy), and to characterise the tumours with the greatest reduction in diagnosis by patient age, sex and tumour stage at diagnosis. We used the health care utilisation databases of the Lombardy region to identify cancer patients' characteristics (eg, sex, age) and cancer-related information (eg, cancer site, stage at diagnosis). The frequency of new cancer diagnoses in 2019 and 2020 were compared in terms of percentage differences in undiagnosed cases. We observed two peaks in the decline in cancer diagnoses: March to May 2020 (-37%) and October to December 2020 (-19%). The decline persisted over the course of 2020 and was higher in males and patients aged 74+. Diagnoses of all four common cancers analysed (female breast, lung, colorectal and prostate) remained below pre-pandemic levels. For breast and colorectal cancers, the decline in diagnoses was high in the age groups targeted by population-based screening programmes. We observed a reduction in localised stage cancer diagnoses for all four cancers. Our data confirm that timely monitoring of cancer diagnoses and interventions to prevent disruption of routine diagnostic services are needed to mitigate the impact of emergencies on cancer patients., (© 2022 The Authors. International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Rare cancers are malignant tumors with an incidence lower than 6/100,000/year at the EU level. Even if more conservative in comparison to the definition used for rare diseases (also from the regulatory point of view), rare cancers defined this way make up more than twenty percent of new cases of malignant tumors in the EU, including Italy. The rarity of a tumor implies that clinical decision-making is more problematic outside reference centers and networks, that available evidence suffers from a higher uncertainty, that organization of health care is more difficult. In 2019, a Joint Action on rare cancers launched by the European Union, run in parallel to a Joint Action on rare diseases, worked out ten categories of recommendations, among which some on the methodology of clinical research and regulatory aspects. In general, it stressed the value of collaborative clinical networks (at the EU level and, more importantly, nationally) around centers of expertise. Working according to a "hub-and-spoke" logic, these networks can improve quality of care while rationalizing health migration. Regarding the regulatory aspects and more in general to the definition of the state of art, in rare cancers the magnitude of clinical benefit should be the same as for common conditions, but the quality of evidence should be accepted to be less stringent in terms of statistical precision. Otherwise, rare cancer patients would be discriminated against in their access to innovative diagnostic and therapeutic options, even when available. Specifically, orphan drugs can suffer from lower interest of pharmaceutical industry. This justifies some privileges they are granted, among which the 10-year market exclusivity is crucial. However, a critical aspect is the perception of the regulatory risk by the industry. This problem could be limited by closer cooperation between regulatory bodies and rare cancer communities and by flexible regulatory mechanisms, in particular in partnership with clinical networks. In Italy, more constructive interpretations of the rules about the compassionate use of drugs, an even application of Law 648 and the future implementation of the new Law on Rare Diseases could help. In general, refining the methodology of clinical research in small samples would be fundamental.

Estimating incidence of rare cancers is challenging for exceptionally rare entities and in small populations. In a previous study, investigators in the Information Network on Rare Cancers (RARECARENet) provided Bayesian estimates of expected numbers of rare cancers and 95% credible intervals for 27 European countries, using data collected by population-based cancer registries. In that study, slightly different results were found by implementing a Poisson model in integrated nested Laplace approximation/WinBUGS platforms. In this study, we assessed the performance of a Poisson modeling approach for estimating rare cancer incidence rates, oscillating around an overall European average and using small-count data in different scenarios/computational platforms. First, we compared the performance of frequentist, empirical Bayes, and Bayesian approaches for providing 95% confidence/credible intervals for the expected rates in each country. Second, we carried out an empirical study using 190 rare cancers to assess different lower/upper bounds of a uniform prior distribution for the standard deviation of the random effects. For obtaining a reliable measure of variability for country-specific incidence rates, our results suggest the suitability of using 1 as the lower bound for that prior distribution and selecting the random-effects model through an averaged indicator derived from 2 Bayesian model selection criteria: the deviance information criterion and the Watanabe-Akaike information criterion., (© The Author(s) 2022. Published by Oxford University Press on behalf of the Johns Hopkins Bloomberg School of Public Health.)

Background: Evidence about late effects in adolescent and young adult (AYA) cancer survivors is scarce. This study assessed the risk of subsequent malignant neoplasms (SMNs) to identify the most common SMNs to be considered in follow-up care., Methods: Population-based cancer registries retrospectively identified first primary tumors (between 1976 and 2013) and SMNs in AYAs (15-39 years old at their cancer diagnosis). AYA cancer survivors were those alive at least 5 years after their first cancer diagnosis. The excess risk of SMNs was measured as standardized incidence ratios (SIRs) and absolute excess risk together with the cumulative incidence of SMNs., Results: The cohort included 67,692 AYA cancer survivors. The excess risk of developing any SMN (SIR, 1.6; 95% confidence interval, 1.5-1.7) was 60%. The excess risk of SMNs was significantly high for survivors of lymphomas; cancers of the breast, thyroid, female genital tract, digestive organs, gonads, and urinary tract; and melanomas. The cumulative incidence of all SMNs in AYA cancer survivors within 25 years of their first cancer diagnosis was approximately 10%. Subsequent tumors contributing to approximately 60% of all SMNs were breast cancer, colorectal cancer, corpus uteri cancer, and ovarian cancer in females and colorectal cancer, bladder cancer, prostate cancer, lung cancer, and lymphomas in males., Conclusions: These results highlight the need to personalize follow-up strategies for AYA cancer survivors., (© 2021 American Cancer Society.)

Background: We described the demographics, cancer subtypes, comorbidities, and outcomes of patients with a history of cancer and coronavirus disease 2019 (COVID-19). Second, we compared patients hospitalized with COVID-19 to patients diagnosed with COVID-19 and patients hospitalized with influenza., Methods: We conducted a cohort study using eight routinely collected health care databases from Spain and the United States, standardized to the Observational Medical Outcome Partnership common data model. Three cohorts of patients with a history of cancer were included: (i) diagnosed with COVID-19, (ii) hospitalized with COVID-19, and (iii) hospitalized with influenza in 2017 to 2018. Patients were followed from index date to 30 days or death. We reported demographics, cancer subtypes, comorbidities, and 30-day outcomes., Results: We included 366,050 and 119,597 patients diagnosed and hospitalized with COVID-19, respectively. Prostate and breast cancers were the most frequent cancers (range: 5%-18% and 1%-14% in the diagnosed cohort, respectively). Hematologic malignancies were also frequent, with non-Hodgkin's lymphoma being among the five most common cancer subtypes in the diagnosed cohort. Overall, patients were aged above 65 years and had multiple comorbidities. Occurrence of death ranged from 2% to 14% and from 6% to 26% in the diagnosed and hospitalized COVID-19 cohorts, respectively. Patients hospitalized with influenza ( n = 67,743) had a similar distribution of cancer subtypes, sex, age, and comorbidities but lower occurrence of adverse events., Conclusions: Patients with a history of cancer and COVID-19 had multiple comorbidities and a high occurrence of COVID-19-related events. Hematologic malignancies were frequent., Impact: This study provides epidemiologic characteristics that can inform clinical care and etiologic studies., (©2021 The Authors; Published by the American Association for Cancer Research.)

Geographical variability of cancer burden was almost exclusively estimated for common cancers. Since rare cancers (RC) have become an area of priority for basic and clinical research and public health organizations, this paper provides, using a common methodology, a detailed comparison of incidence and survival for RC in the US and Europe. We estimated incidence and net survival of 199 malignant RC from data of 2 580 000 patients collected by 18 US-SEER and 94 European registries, diagnosed within the most recent common period 2000-2007. RC were defined according to the criterion of crude annual incidence rates <6/100 000. In total, 196 RC were classified as rare in both populations. Of these, 43 had incidence rates significantly different by at least 0.2 per 100 000:34 higher in the US and 9 higher in Europe. Five-year net survival for all RC combined significantly differed: 54% in the US and 48% in Europe. Survival for 62 RC was significantly higher in the US vs 6 higher in Europe. Differences were not concentrated in a particular cancer family, and were mostly relevant for cases diagnosed >65+ years of age. Use of standardized methods evidenced that incidence and survival rate of majority of RC were higher in the United States compared to Europe. Possible reasons for such differences, requiring further studies, include distribution of risk factors, ability to diagnose RC, different registration practices, and use of updated International Classification of Diseases for Oncology., (© 2020 The Authors. Cancer Medicine published by John Wiley & Sons Ltd.)

Introduction: Epidemiologic information on rare cancers is scarce outside of the Western countries. The project "surveillance of rare cancers in Asia" (RARECAREnet Asia) provides, for the first time, the burden of rare cancers in some Asian countries based on the latest list., Objectives: 1) to assess whether the European list of rare cancers fits the Asian setting and 2) to compare the incidences of rare cancers between Europe and Asian countries., Material and Methods: Population-based cancer registry data on patients diagnosed from 2011 to 2015 in Japan, Korea, and Taiwan and patients diagnosed from 2000 to 2007 in 94 European registries were analysed. The incidences for all cancers were calculated; they were then grouped into several tiers and families according to the rare cancer list, and whether cancers rare was examined., Results: Rare cancer counts according to the list in the observed population were 196 in Japan, 203 in Korea, 198 in Taiwan, and 198 in the EU. The proportions of rare in overall incidence were 16.3% in Japan, 23.7% in Korea, 24.2% in Taiwan, and 22.2% in the EU. The numbers of newly diagnosed rare cancer cases in 2015 were 140,188 in Japan, 52,071 in Korea, and 24,147 in Taiwan., Conclusion: Most rare cancers in Europe were also rare in the Asian countries considered. The observed differences were due to well-known risk factors. The European definition and list of rare cancers appear to reflect well cancer incidence in East Asia., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: The Surveillance of Rare Cancers in Europe (RARECARE) project proposed a definition and a list of rare cancers. The Joint Action on Rare Cancers (JARC), launched by the European Union and involving 18 member states and 34 partners, promoted a wide consensus effort to review the list., Patients and Methods: A group of experts was set up, including scientific societies, member state representatives of JARC, representatives of the European Reference Networks dedicated to rare cancers and rare cancer patient advocates. The definition and the list of rare clinical entities, based on the incidence data provided by two European projects (RARECARE and RARECAREnet), were rediscussed through a consensus meeting of the expert panel., Results: By consensus, it was reiterated that the best criterion for a definition of rare cancers is incidence, rather than prevalence. By consensus, the experts slightly modified the composition of the tiers of rare cancers, according to the definition based on an incidence threshold <6/100 000/year, and grouped all rare cancers within 12 families of rare cancers. Even when defined conservatively this way, rare cancers are not rare collectively, since they correspond to 10%-20% of all cancer cases., Conclusions: The list of rare cancers reviewed by JARC should be viewed as a tool in the fight against rare cancers and rare diseases. It may help to appreciate that rare cancers are cancers and rare diseases at the same time, combining issues and difficulties of both. We hope that refinements to the list and a wider understanding of its implications may contribute to increase awareness of problems posed by rare cancers and to improve quality of care in a large group of patients with cancer, who may be discriminated against just because of the low frequency of their diseases., Competing Interests: Competing interests: PGC: honoraria for advisory/speaker’s role from Deciphera Pharmaceuticals, Eisai, Eli Lilly, Nektar Ther, Pfizer, PharmaMar; institutional fundings from Amgen Dompé, AROG, Bayer, Blueprint, Eli Lilly, Daiichi Sankyo Pharma, Epizyme, Glaxo SK, Novartis, Pfizer, PharmaMar., (© Author (s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. Published by BMJ on behalf of the European Society for Medical Oncology.)

Background: The proportion of patients cured of cancer is usually estimated with cure models assuming they have the same death risk as the general population. These patients, even when cured, often maintain an extra death risk compared to the overall population. Our aims were to estimate this extra risk, and to take it into account in estimating cure proportions and relative survival (RS)., Methods: We used RS mixture model with an additional parameter expressing the extra noncancer death risk of patients, assumed constant with age. We applied the model to the SEER registries survival data (1990-1994 diagnosed patients) with colorectal, breast, and lung cancers, and followed up to 2013., Results: The estimated relative risk of death for cured patients versus the general population was 1.11 for colorectal, 1.16 for breast, and 2.17 and 2.12, respectively, for female and male lung cancers. Taking this extra risk into account leads, for all cancers, to a higher estimated proportion of cured and a lower RS of uncured patients. In addition, it leads to a higher estimated RS for all patients aged >70 years, and for lung cancer patients aged >50 years, at diagnosis., Conclusions: Mortality of survivors not directly due to the diagnosed cancer was significantly higher than in the general population. It affected the estimates of cure proportions for all age classes and RS in the elderly.

Although all tumours are rare in childhood, there are some particularly rare paediatric cancers which have not benefited from advances made by the international paediatric oncology network. To establish a shared definition and produce a list of these entities, the European Union Joint Action on Rare Cancers (JARC) promoted a consensus effort. The definition was based on the incidence rates estimated using the information network on rare cancers (RARECAREnet) database, pooling data from 94 population-based cancer registries and 27 countries. The RARECAREnet list of cancers was used to estimate the incidence rates. This list groups cancers by combining the International Classification of Diseases for Oncology, third edition, morphology and topography codes. According to the consensus, very rare paediatric cancers were identified as those with an annual incidence <2/1000000 and corresponded to 11% of all cancers in patients aged 0-14 years. Two subgroups were identified: tumour types typical of childhood (i.e. hepatoblastoma, pleuropulmonary blastoma, pancreatoblastoma) and those typical of adult age (i.e. carcinomas, melanoma). The threshold of 2/1000000 could also be adopted in populations aged 0-19 years: in this case, three tumour types had an incidence rate which was >2/1000000 (i.e. thyroid and testicular cancers and skin melanoma), but the consensus experts considered them as 'very rare' according to their clinical needs (e.g. shortage of knowledge and clinical expertise as the other rare paediatric cancers). The JARC consensus produced a definition and a list of very rare paediatric cancers which may represent a starting point for prioritising research on these tumours, based on data and patients' clinical needs., (Copyright © 2019 Elsevier Ltd. All rights reserved.)

Introduction:: Rare cancers are a challenge for clinical practice as well as for epidemiology and public health. Studies on this subject are few and limited to the study of cases with scarce epidemiologic information. This study aimed to evaluate the incidence of rare cancers and to compare the demographic, anatomic, and histologic characteristics of rare and nonrare (common) cancers., Methods:: Incidence data were obtained from the Population-based Cancer Registry of São Paulo, Brazil. Rare neoplasms were those defined in the RARECARE list, which takes into account an incidence lower than 6/100,000/year., Results:: In São Paulo, 20.4% of tumors had an incidence lower than 6/100,000/year from 1997 to 2012, being therefore considered as rare tumors. We identified 11 entities with an incidence greater than 6/100,000/year (common neoplasms) and 186 entities with an incidence lower than 6/100,000/year (rare neoplasms). The mean annual incidence of all cancers was 365 per 100,000 in São Paulo between 1997 and 2012, and the incidence of all rare tumors was 74.5 per 100,000., Conclusions:: This study presents the burden of rare cancers in Brazil. It is expected to be an incentive for further studies of these entities in order to know the epidemiologic profile of rare tumors in Brazil and to provide a more effective diagnostic and therapeutic approach.

Rare cancers represent approximately one fourth of all cancers. Despite being a heterogeneous group of diseases, they share similar problems including lack of expertise, issues in quality of care, discrepancies in outcome and limitations in research. Traditionally, centralization of rare cancer patients to dedicated reference centres has been recommended to ensure expertise, multidisciplinarity and access to innovation. However, centralization entails health migration, rationing of resources and a potential failure in routine care. By ensuring appropriate care to all patients regardless the point of access, networking seems the most appropriate answer to the problem of rare cancers. The launch of the Joint Action on Rare Cancers as well as the recent establishment of the European Reference Networks represent for the first time a concrete opportunity to make networking a reality and ultimately reduce disparities and improve outcome in these diseases., (Copyright © 2018 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Cancer burden in adolescents and young adults (AYAs) is expressed through a large proportion of the quality of life lost on individual level and also causes losses to the society in terms of a decreased productivity and social structure. A specific cancer spectrum and distinctive needs of AYA patients require targeted studies and cancer control measures. Incidence is intermediate between that for children and for older adults, and two-thirds of the AYA cancers affect women. Cancers of the breast and cervix uteri, representing a large portion of the burden, are amenable to prevention. Survival is relatively high, but it is lower in AYA patients with certain cancers that are common in childhood or older adulthood. Tailored cancer care with centralized multidisciplinary provision improves the outcome, as demonstrated by survival of leukemia patients. Mortality is decreasing in high-income countries for the cancers that contribute to the burden most, but lack of progress is seen for some rarer subtypes, such as brain tumors and sarcomas of the bone and soft tissue. There is unacceptable lack of information on cancer burden in low-income countries in which the outcomes for AYA patients are likely dreadful. Investment is required to establish cancer registration system and appropriate cancer care delivery in these settings.

Background: The RARECAREnet project has updated the estimates of the burden of the 198 rare cancers in each European country. Suspecting that scant data could affect the reliability of statistical analysis, we employed a Bayesian approach to estimate the incidence of these cancers., Methods: We analyzed about 2,000,000 rare cancers diagnosed in 2000-2007 provided by 83 population-based cancer registries from 27 European countries. We considered European incidence rates (IRs), calculated over all the data available in RARECAREnet, as a valid a priori to merge with country-specific observed data. Therefore we provided (1) Bayesian estimates of IRs and the yearly numbers of cases of rare cancers in each country; (2) the expected time (T) in years needed to observe one new case; and (3) practical criteria to decide when to use the Bayesian approach., Results: Bayesian and classical estimates did not differ much; substantial differences (>10%) ranged from 77 rare cancers in Iceland to 14 in England. The smaller the population the larger the number of rare cancers needing a Bayesian approach. Bayesian estimates were useful for cancers with fewer than 150 observed cases in a country during the study period; this occurred mostly when the population of the country is small., Conclusion: For the first time the Bayesian estimates of IRs and the yearly expected numbers of cases for each rare cancer in each individual European country were calculated. Moreover, the indicator T is useful to convey incidence estimates for exceptionally rare cancers and in small countries; it far exceeds the professional lifespan of a medical doctor., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Cancer survival has persistently been shown to be worse for Eastern European and UK/Ireland patients than those of other European regions. This is often attributed to later stage at diagnosis. However, few stage-specific survival comparisons are available, so it is unclear whether poorer quality treatment or other factors also contribute. For the first time, European cancer registries have provided stage-at-diagnosis data to EUROCARE, enabling population-based stage-specific survival estimates across Europe., Data and Methods: In this retrospective observational study, stage at diagnosis (as TNM, condensed TNM, or Extent of Disease) was analysed for patients (≥15 years) from 15 countries grouped into 4 regions (Northern Europe: Norway; Central Europe: Austria, France, Germany, Switzerland, The Netherlands; Southern Europe: Croatia, Italy, Slovenia, and Spain; and Eastern Europe: Bulgaria, Estonia, Lithuania, Poland, and Slovakia), diagnosed with 7 malignant cancers in 2000-2007, and followed to end of 2008. A new variable (reconstructed stage) was created which used all available stage information. Age-standardised 5-year relative survival (RS) by reconstructed stage was estimated and compared between regions. Excess risks of cancer death in the 5 years after diagnosis were also estimated, taking age, sex and stage into account., Results: Low proportions of Eastern European patients were diagnosed with local stage cancers and high proportions with metastatic stage cancers. Stage-specific RS (especially for non-metastatic disease) was generally lower for Eastern European patients. After adjusting for age, sex, and stage, excess risks of death remained higher for Eastern European patients than for European patients in general., Conclusions: Late diagnosis alone does not explain worse cancer survival in Eastern Europe: greater risk of cancer death together with worse stage-specific survival suggest less effective care, probably in part because fewer resources are allocated to health care than in the rest of Europe. We recommend that Eastern European cancer registries and other involved bodies to draw attention to poor cancer survival, so as to stimulate research and inform policies to improve outcomes., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Cancer registries (CRs) are fundamental for estimating cancer burden, evaluating screening and monitoring health service performance. Stage at diagnosis-an essential information item collected by CRs-has been made available, for the first time, by CRs participating in EUROCARE-5. We analysed the quality of this information and estimated stage-specific survival across Europe for CRs with good data quality., Data and Methods: Sixty-two CRs sent stage (as TNM, condensed TNM or extent of disease) for 15 cancers diagnosed in 2000-2007. We assessed the quality, partly by comparing stage according to the three systems. We also developed procedures to reconstruct stage (categories: local, regional, metastatic and unknown) using information from all three systems, thus minimising the amount of missing information., Results: Moderate-to-excellent stage concordance was found for practically all 24 CRs, for which it was possible to compare at least two staging systems. However, since stage was often incorrectly assigned, and information on the presence/absence of metastases was often lacking, data on only 7/15 cancers from 34/62 CRs (15 countries) were of sufficient quality for further analysis. Cases diagnosed ≥70 years had more advanced (or lacking) stage- and worse stage-specific survival than those <70 years., Conclusions: Many European CRs collect and record reasonably accurate stage information. Others have difficulties. Both the completeness of primary data and the accuracy of stage coding need to be improved in order for CRs to fulfil their expanding roles in cancer control. We propose our stage reconstruction/checking procedures as a means of fully exploiting the stage information provided by EUROCARE CRs. More advanced (or lacking) stage at diagnosis plus poorer stage-specific survival in the elderly are worrying., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Background: Rare cancers pose challenges for diagnosis, treatments, and clinical decision making. Information about rare cancers is scant. The RARECARE project defined rare cancers as those with an annual incidence of less than six per 100 000 people in European Union (EU). We updated the estimates of the burden of rare cancers in Europe, their time trends in incidence and survival, and provide information about centralisation of treatments in seven European countries., Methods: We analysed data from 94 cancer registries for more than 2 million rare cancer diagnoses, to estimate European incidence and survival in 2000-07 and the corresponding time trends during 1995-2007. Incidence was calculated as the number of new cases divided by the corresponding total person-years in the population. 5-year relative survival was calculated by the Ederer-2 method. Seven registries (Belgium, Bulgaria, Finland, Ireland, the Netherlands, Slovenia, and the Navarra region in Spain) provided additional data for hospitals treating about 220 000 cases diagnosed in 2000-07. We also calculated hospital volume admission as the number of treatments provided by each hospital rare cancer group sharing the same referral pattern., Findings: Rare cancers accounted for 24% of all cancers diagnosed in the EU during 2000-07. The overall incidence rose annually by 0.5% (99·8% CI 0·3-0·8). 5-year relative survival for all rare cancers was 48·5% (95% CI 48·4 to 48·6), compared with 63·4% (95% CI 63·3 to 63·4) for all common cancers. 5-year relative survival increased (overall 2·9%, 95% CI 2·7 to 3·2), from 1999-2001 to 2007-09, and for most rare cancers, with the largest increases for haematological tumours and sarcomas. The amount of centralisation of rare cancer treatment varied widely between cancers and between countries. The Netherlands and Slovenia had the highest treatment volumes., Interpretation: Our study benefits from the largest pool of population-based registries to estimate incidence and survival of about 200 rare cancers. Incidence trends can be explained by changes in known risk factors, improved diagnosis, and registration problems. Survival could be improved by early diagnosis, new treatments, and improved case management. The centralisation of treatment could be improved in the seven European countries we studied., Funding: The European Commission (Chafea)., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Rare adult cancer (RAC) is characterised by an incidence of less than six cases per 100,000 people per annum; 4,300,000 patients in the European Union are living with rare cancer (22% of all new human cancers). These cancers are linked with worse survival rates than 'frequent' tumours (5-year survival: 47% for RAC against 65% for 'common' cancers), mainly because of: (1) delays in obtaining an accurate diagnosis, (2) inadequate treatments given in curative phases and (3) restricted opportunities for patients to participate in clinical trials because of the lack of support for dedicated trials for this disease group from both academic and industrial sponsors. Although quantitative studies to measure the socioeconomic burden of RACs as a whole are still lacking, the increasing fragmentation of all cancers into molecular subgroups implies a substantial increase in the number of RACs and their associated socioeconomic burden. To answer this urgent and growing need, some countries, cooperative groups, and cancer institutes delineated national and/or regional organisations to promote quality management for RACs. Currently, the European Union (EU) is supporting an official EU call to organise a European network dedicated to RACs. The goals will be to pool the vast knowledge and expertise of the 67 EU clinical reference centres and to cover ten rare adult solid cancer domains across more than 18 countries in order to deploy an integrated, EU-wide capacity towards accelerated innovative treatments and care for RACs while empowering patients. This article will summarise these experiences and the potential benefit for patients., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Purpose: Rare cancers represent 22% of all tumors in Europe; however, the quality of the data of rare cancers may not be as good as the quality of data for common cancer. The project surveillance of rare cancers in Europe (RARECARE) had, among others, the objective of assessing rare cancer data quality in population-based cancer registries (CRs). Eight rare cancers were considered: mesothelioma, liver angiosarcoma, sarcomas, tumors of oral cavity, CNS tumors, germ cell tumors, leukemia, and malignant digestive endocrine tumors., Methods: We selected data on 18,000 diagnoses and revised, on the basis of the pathologic and clinical reports (but not on pathologic specimens), unspecified morphology and topography codes originally attributed by CR officers and checked the quality of follow-up of long-term survivors of poor prognosis cancers., Results: A total of 38 CRs contributed from 13 European countries. The majority of unspecified morphology and topography cases were confirmed as unspecified. The few unspecified cases that, after the review, changed to a more specific diagnosis increased the incidence of the common cancer histotypes. For example, 11% of the oral cavity epithelial cancers were reclassified from unspecified to more specific diagnoses: 8% were reclassified as squamous cell carcinoma (commoner) and only 1% as adenocarcinoma (rarer). The revision confirmed the majority of long-term survivors revealing a relative high proportion of mesothelioma long-term survivors. The majority of appendix carcinoids changed behavior from malignant to borderline lesions., Conclusions: Our study suggests that the problem of poorly specified morphology and topography cases is mainly one of difficulty in reaching a precise diagnosis. The awareness of the importance of data quality for rare cancers should increase among registrars, pathologists, and clinicians.

Background: Data from EUROCARE have consistently shown lower survival for adolescents and young adults (AYAs; aged 15-24 years) than for children (0-14 years) for most cancers that affect both groups, and modest survival improvements up to 2000-02. AYAs have longer survival than that of adults for most cancers. We used the latest definition of AYAs (aged 15-39 years) and provided estimates of 5-year relative survival for European AYAs with cancer diagnosed in 2000-07, compared with children and adults (40-69 years) with cancer, and assessed survival improvements over time., Methods: We analysed data from population-based cancer registries of 27 European countries participating in EUROCARE-5. We used the so-called complete method to estimate 5-year, population-weighted relative survival for 19 cancers affecting AYAs and children, and for 27 cancers affecting AYAs and adults. We assessed relative-survival differences between children versus AYAs, and between AYAs versus adults, using the Z test. We used the period approach to estimate 5-year relative survival over time for children and AYAs, and used a generalised linear model to model survival time trends (1999-2007) and to assess the significance of changes over time., Findings: We analysed 56 505 cancer diagnoses in children, 312 483 in AYAs, and 3 567 383 in adults. For all cancers combined, survival improved over time for AYAs (from 79% [95% CI 78·1-80·5] in 1999-2002 to 82% [81·1-83·3] in 2005-07; p<0·0001) and children (from 76% [74·7-77·1] to 79% [77·2-79·4]; p<0·0001). Survival improved significantly in children and AYAs for acute lymphoid leukaemia (p<0·0001) and non-Hodgkin lymphoma (p<0·0001 in AYAs and p=0·023 in children). Survival improved significantly in AYAs only for CNS tumours (p=0·0046), astrocytomas (p=0·040), and malignant melanomas (p<0·0001). Survival remained significantly worse in AYAs than in children for eight important cancers: acute lymphoid leukaemias, acute myeloid leukaemias, Hodgkin's lymphomas, non-Hodgkin lymphomas, astrocytomas, Ewing's sarcomas, and rhabdomyosarcomas (p<0·0001 in all cases), and osteosarcomas (p=0·011)., Interpretation: Notwithstanding the encouraging results for some cancers, and overall, we showed poorer survival in AYAs than in children for the eight important cancers. Recent European initiatives to improve outcomes in AYAs might reduce the survival gap between children and AYAs, but this reduction can only be verified by future population-based studies., Funding: Italian Ministry of Health, European Commission., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

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.

Background: Survival and cure rates for childhood cancers in Europe have greatly improved over the past 40 years and are mostly good, although not in all European countries. The EUROCARE-5 survival study estimates survival of children diagnosed with cancer between 2000 and 2007, assesses whether survival differences among European countries have changed, and investigates changes from 1999 to 2007., Methods: We analysed survival data for 157,499 children (age 0-14 years) diagnosed between Jan 1, 1978 and Dec 31, 2007. They came from 74 population-based cancer registries in 29 countries. We calculated observed, country-weighted 1-year, 3-year, and 5-year survival for major cancers and all cancers combined. For comparison between countries, we used the corrected group prognosis method to provide survival probabilities adjusted for multiple confounders (sex, age, period of diagnosis, and, for all cancers combined without CNS cancers, casemix). Age-adjusted survival differences by area and calendar period were calculated with period analysis and were given for all cancers combined and the major cancers., Findings: We analysed 59,579 cases. For all cancers combined for children diagnosed in 2000-07, 1-year survival was 90.6% (95% CI 90.2-90.9), 3-year survival was 81.0 % (95% CI 80.5-81.4), and 5-year survival was 77.9% (95% CI 77.4-78.3). For all cancers combined, 5-year survival rose from 76.1% (74.4-77.7) for 1999-2001, to 79.1% (77.3-80.7) for 2005-07 (hazard ratio 0.973, 95% CI 0.965-0.982, p<0.0001). The greatest improvements were in eastern Europe, where 5-year survival rose from 65.2% (95% CI 63.1-67.3) in 1999-2001, to 70.2% (67.9-72.3) in 2005-07. Europe-wide average yearly change in mortality (hazard ratio) was 0.939 (95% CI 0.919-0.960) for acute lymphoid leukaemia, 0.959 (0.933-0.986) for acute myeloid leukaemia, and 0.940 (0.897-0.984) for non-Hodgkin lymphoma. Mortality for all of Europe did not change significantly for Hodgkin's lymphoma, Burkitt's lymphoma, CNS tumours, neuroblastoma, Wilms' tumour, Ewing's sarcoma, osteosarcoma, and rhabdomyosarcoma. Disparities for 5-year survival persisted between countries and regions, ranging from 70% to 82% (for 2005-07)., Interpretation: Several reasons might explain persisting inequalities. The lack of health-care resources is probably most important, especially in some eastern European countries with limited drug supply, lack of specialised centres with multidisciplinary teams, delayed diagnosis and treatment, poor management of treatment, and drug toxicity. In the short term, cross-border care and collaborative programmes could help to narrow the survival gaps in Europe., Funding: Italian Ministry of Health, European Commission, Compagnia di San Paolo Foundation., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: Cancer survival is a key measure of the effectiveness of health-care systems. EUROCARE-the largest cooperative study of population-based cancer survival in Europe-has shown persistent differences between countries for cancer survival, although in general, cancer survival is improving. Major changes in cancer diagnosis, treatment, and rehabilitation occurred in the early 2000s. EUROCARE-5 assesses their effect on cancer survival in 29 European countries., Methods: In this retrospective observational study, we analysed data from 107 cancer registries for more than 10 million patients with cancer diagnosed up to 2007 and followed up to 2008. Uniform quality control procedures were applied to all datasets. For patients diagnosed 2000-07, we calculated 5-year relative survival for 46 cancers weighted by age and country. We also calculated country-specific and age-specific survival for ten common cancers, together with survival differences between time periods (for 1999-2001, 2002-04, and 2005-07)., Findings: 5-year relative survival generally increased steadily over time for all European regions. The largest increases from 1999-2001 to 2005-07 were for prostate cancer (73.4% [95% CI 72.9-73.9] vs 81.7% [81.3-82.1]), non-Hodgkin lymphoma (53.8% [53.3-54.4] vs 60.4% [60.0-60.9]), and rectal cancer (52.1% [51.6-52.6] vs 57.6% [57.1-58.1]). Survival in eastern Europe was generally low and below the European mean, particularly for cancers with good or intermediate prognosis. Survival was highest for northern, central, and southern Europe. Survival in the UK and Ireland was intermediate for rectal cancer, breast cancer, prostate cancer, skin melanoma, and non-Hodgkin lymphoma, but low for kidney, stomach, ovarian, colon, and lung cancers. Survival for lung cancer in the UK and Ireland was much lower than for other regions for all periods, although results for lung cancer in some regions (central and eastern Europe) might be affected by overestimation. Survival usually decreased with age, although to different degrees depending on region and cancer type., Interpretation: The major advances in cancer management that occurred up to 2007 seem to have resulted in improved survival in Europe. Likely explanations of differences in survival between countries include: differences in stage at diagnosis and accessibility to good care, different diagnostic intensity and screening approaches, and differences in cancer biology. Variations in socioeconomic, lifestyle, and general health between populations might also have a role. Further studies are needed to fully interpret these findings and how to remedy disparities., Funding: Italian Ministry of Health, European Commission, Compagnia di San Paolo Foundation, Cariplo Foundation., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

This paper describes the usage and the performance evaluation of the completeness index method in the 'Surveillance of Rare Cancers in Europe project' (RARECARE) for estimating rare cancer prevalence in Europe. The 15-year prevalence at 1st January 2003 for 255 cancers is obtained from a pool of 22 RARECARE cancer registries (CRs). Incidence and survival models are applied to the RARECARE database to estimate the parameters from which the completeness indices are calculated. Complete prevalence is obtained adjusting the observed 15-year prevalence by the completeness index, to account for those cancer survivors diagnosed before the CR activity started. Main factors influencing the performance of the completeness index method for rare cancers are the same as for common cancers: age distribution of incidence and lethality of the cancer. For cancers occurring in the elderly, with low survival rates and consequently a restricted number of long-term survivors we obtained completeness indices higher than 0.9. Values lower than 0.7 correspond to those cancers with good prognosis and/or incidence more concentrated at the younger ages, indicating that 15 years of follow up are insufficient to detect all prevalent cases. Validation analysis shows that for a restricted subgroup of rare cancers with very low incidence and low survival, the completeness indices were not able to adequately correct the observed prevalence even considering a registration period of 20 years. On average, sensitivity analyses show a slight overestimation of complete prevalence for rare and common cancers whose increasing incidence is known in literature. RARECARE is the largest project on rare cancers conducted to date. Improving health care programs for cancer survivors is a public health priority and prevalence data which provides important information in this field should be regularly asked to Member States and included in the EU health statistics., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Cancer survival varies markedly across Europe. We analyzed variations in all-cancer 5-year relative survival in relation to macroeconomic and health-care indicators, and 5-year relative survival for three major cancers (colorectal, prostate, breast) in relation to application of standard treatments, to serve as baseline for monitoring the efficacy of new European initiatives to improve cancer survival. Five-year relative survival data were from the European cancer registry-based study of cancer patients' survival and care (EUROCARE-4). Macroeconomic and health system data were from the Organisation for Economic Co-operation and Development, and European Observatory on Health Care Systems. Information on treatments given was from EUROCARE studies. Total national health spending varied widely across Europe and correlated linearly with survival (R = 0.8). Countries with high spending had high numbers of diagnostic and radiotherapy units, and 5-year relative survival was good (>50%). The treatments given for major cancers also varied; advanced stage at diagnosis was associated with poor 5-year relative survival and low odds of receiving standard treatment for breast and colorectal cancer.

Aims and Background: The project Surveillance of rare cancers in Italy (RITA) provides, for the first time, estimates of the burden of rare cancers in Italy based on the list of rare cancers proposed in collaboration with the European project Surveillance of Rare Cancers in Europe (RARECARE)., Methods: RITA analyzed data from Italian population-based cancer registries (CR). The period of diagnosis was 1988 to 2002, and vital status information was available up to December 31, 2003. Incidence rates were estimated for the period 1995-2002, survival for the years 2000-2002 (with the period method of Brenner), and complete prevalence at January 1, 2003., Results: Rare cancers are those with an incidence <6/100,000/year. In Italy, every year there are 60,000 new diagnoses of rare cancers corresponding to 15% of all new cancer diagnoses. Five-year relative survival was on the average worse for rare cancers (53%) than for common cancers (73%). A total of 770,000 patients were living in Italy in 2008 with a diagnosis of a rare cancer, 22% of the total cancer prevalence., Conclusions: Our estimates constitute a useful base for further research and support the idea that rare cancers are a public health problem that deserves attention. Centers of expertise for rare cancers that pool cases, expertise and resources could ensure an adequate clinical management for these diseases. Our data also showed that cancer registries are suitable sources of data to estimate incidence, prevalence and survival for rare cancers and should continue to monitoring rare cancers in Italy.

Purpose: Epidemiologic information on rare cancers is scarce. The project Surveillance of Rare Cancers in Europe (RARECARE) provides estimates of the incidence, prevalence and survival of rare cancers in Europe based on a new and comprehensive list of these diseases., Materials and Methods: RARECARE analysed population-based cancer registry (CR) data on European patients diagnosed from 1988 to 2002, with vital status information available up to 31st December 2003 (latest date for which most CRs had verified data). The mean population covered was about 162,000,000. Cancer incidence and survival rates for 1995-2002 and prevalence at 1st January 2003 were estimated., Results: Based on the RARECARE definition (incidence <6/100,000/year), the estimated annual incidence rate of all rare cancers in Europe was about 108 per 100,000, corresponding to 541,000 new diagnoses annually or 22% of all cancer diagnoses. Five-year relative survival was on average worse for rare cancers (47%) than common cancers (65%). About 4,300,000 patients are living today in the European Union with a diagnosis of a rare cancer, 24% of the total cancer prevalence., Conclusion: Our estimates of the rare cancer burden in Europe provide the first indication of the size of the public health problem due to these diseases and constitute a useful base for further research. Centres of excellence for rare cancers or groups of rare cancers could provide the necessary organisational structure and critical mass for carrying out clinical trials and developing alternative approaches to clinical experimentation for these cancers., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

The burden of rare tumors in Europe is still unknown and no generally accepted definition of them exist. The Surveillance of Rare Cancers in Europe project (funded by the European Commission) aimed at providing a definition of "rare cancer", a list of cancers and rare cancer burden indicators, based on population-based cancer registry data, across Europe. An international consensus group agreed that incidence is the most appropriate indicator for measuring rare cancers frequency and set the threshold for rarity at 6/100,000/year. The list of rare cancers was based on the International Classification of Diseases for Oncology (ICD-O 3rd edition) and it was hierarchically structured in 2 layers based on various combinations of ICD-O morphology and topography codes: layer (1) families of tumors (relevant for the health care organisation) and layer (2) tumors clinically meaningful (relevant for clinical decision making and research). The burden indicators were estimated and are provided in this chapter.

Abstract Germ-cell tumours (GCT) of the testis and penile squamous cell carcinoma (PeSCC) are a rare and a very rare uro-genital cancers, respectively. Both tumours are well defined entities in terms of management, where specific recommendations - in the form of continuously up-to-dated guide lines-are provided. Impact of these tumour is relevant. Testicular GCT affects young, healthy men at the beginning of their adult life. PeSCC affects older men, but a proportion of these patients are young and the personal consequences of the disease may be devastating. Deviation from recommended management may be a reason of a significant prognostic worsening, as proper treatment favourably impacts on these tumours, dramatically on GCT and significantly on PeSCC. RARECAREnet data may permit to analyse how survivals may vary according to geographical areas, histology and age, leading to assume that non-homogeneous health-care resources may impact the cure and definitive outcomes. In support of this hypothesis, some epidemiologic datasets and clinical findings would indicate that survival may improve when appropriate treatments are delivered, linked to a different accessibility to the best health institutions, as a consequence of geographical, cultural and economic barriers. Finally, strong clues based on epidemiological and clinical data support the hypothesis that treatment delivered at reference centres or under the aegis of a qualified multi-institutional network is associated with a better prognosis of patients with these malignancies. The ERN EURACAN represents the best current European effort to answer this clinical need. [ABSTRACT FROM AUTHOR]

Introduction Epidemiologic information on rare cancers is scarce out of the Western countries. The project of surveillance of rare cancers in Asian countries (RARECAREnet Asia) provides the first standardized incidence in Asia based on the latest cancer group based on ICD-O-3. Material and methods We analyzed population-based cancer registry data on patients diagnosed from 2011 to 2015 in Japan, Korea and Taiwan in comparison with the data in EU. With recent difficulty of data centralization in international studies, the analysis was performed in each country based on SEER*Stat. Results Data quality in the three Asian countries were as high as EU. Based on the revised RARECARE definition (crude incidence <6/100,000/year in tier-1), the incidence of all rare cancers was 93.9 in Japan, 106.1 in Korea and 103.5 in Taiwan and 67.8 in EU corresponding to 14%, 24.0%, 24% and 11% of all cancer diagnoses. Among rare cancer families, epithelial tumours of nasopharynx, oropharynx and oral cavity and lips were not rare in Taiwan. Epithelial tumours of oral cavity and lip was not rare in Japan, neither. Epithelial tumours of gallbladder and extra hepatic biliary tract was not rare in Japan and Korea. Thyroid cancer was not rare in all three Asian countries. As for the common cancer families, epithelial tumours of esophagus was not common in Korea. Epithelial tumours of corpus uteri was not common in Korea and ovary was not common in Korea and Taiwan. Skin melanoma was not common in Asia. Even among 215 cancer groups in tier-2s, 197 was rare in Japan, 203 was rare in Korea and 201 was rare in Taiwan patients, and 198 in EU. 197 was rare in all the 4 areas in the world. Conclusion Most of the continental gaps found in the current study were due to well known risk factors, and we conclude that EU based RARECARE rarity threshold fits well to cancer incidence in East Asia. [ABSTRACT FROM AUTHOR]