National rare diseases registry in Spain: pilot study of the Spanish Rare Diseases Registries Research Network (SpainRDR)

Supplement 7th European Conference on Rare Diseases and Orphan Products (ERCD 2014)Background The development of a national Rare Diseases (RD) registry in Spain was launched in 2012 with the project SpainRDR, supported by the International Rare Diseases Research Consortium (IRDiRC). SpainRDR includes two different strategies: patient registries addressed to patient outcome research and population-based registries addressed to epidemiologic research, health and social planning [1]. The pilot study aims to detect the difficulties of developing the national and population-based RD registry

Geographical pattern of brain cancer incidence in the Navarre and Basque Country regions of Spain

Background: The study of the distribution of brain cancer mortality in Spain shows a grouping of highest risk provinces corresponding to the autonomous regions of Navarre and the Basque Country. Aim: To explore the possible existence of geographical patterns in these areas. Methods: Municipal maps of brain cancer incidence were drawn up and the influence of land use related variables on the distribution of the disease duly analysed. Autoregressive conditional models were used to plot smoothed municipal maps. The influence of explanatory land use variables, ascertained by remote sensing, was assessed. Results: The maps revealed that certain towns situated in the "Media" and "Cantábrica-Baja Montaña" districts of Navarre were areas of highest risk. Among the towns in question, those in the "Media" district lie very close to the city of Pamplona. However, the pattern of brain cancer incidence in Navarre and the Basque Country could not be conclusively said to be determined by any specific type of land cover and/or crop. Conclusions: Results suggest a possible increase of risk linked to areas devoted to a high percentage of non-irrigated arable land

National Rare Disease Registries: overview from Spain

From 7th European Conference on Rare Diseases and Orphan Products (ECRD 2014).N

International incidence of childhood cancer, 2001-10: a population-based registry study

Background Cancer is a major cause of death in children worldwide, and the recorded incidence tends to increase with time. Internationally comparable data on childhood cancer incidence in the past two decades are scarce. This study aimed to provide internationally comparable local data on the incidence of childhood cancer to promote research of causes and implementation of childhood cancer control. Methods This population-based registry study, devised by the International Agency for Research on Cancer in collaboration with the International Association of Cancer Registries, collected data on all malignancies and non-malignant neoplasms of the CNS diagnosed before age 20 years in populations covered by high-quality cancer registries with complete data for 2001-10. Incidence rates per million person-years for the 0-14 years and 0-19 years age groups were age-adjusted using the world standard population to provide age-standardised incidence rates (WSRs), using the age-specific incidence rates (ASR) for individual age groups (0-14 years, 5-9 years, 10-14 years, and 15-19 years). All rates were reported for 19 geographical areas or ethnicities by sex, age group, and cancer type. The regional WSRs for children aged 0-14 years were compared with comparable data obtained in the 1980s. Findings Of 532 invited cancer registries, 153 registries from 62 countries, departments, and territories met quality standards, and contributed data for the entire decade of 2001-10. 385 509 incident cases in children aged 0-19 years occurring in 2-6 billion person-years were included. The overall WSR was 140.6 per million person-years in children aged 0-14 years (based on 284 649 cases), and the most common cancers were leukaemia (WSR 46.4), followed by CNS tumours (WSR 28.2), and lymphomas (WSR 15.2). In children aged 15-19 years (based on 100 860 cases), the ASR was 185.3 per million person-years, the most common being lymphomas (ASR 41.8) and the group of epithelial tumours and melanoma (ASR 39.5). Incidence varied considerably between and within the described regions, and by cancer type, sex, age, and racial and ethnic group. Since the 1980s, the global WSR of registered cancers in children aged 0-14 years has increased from 124.0 (95% CI 123.3-124.7) to 140.6 (140.1-141.1) per million person-years. Interpretation This unique global source of childhood cancer incidence will be used for aetiological research and to inform public health policy, potentially contributing towards attaining several targets of the Sustainable Development Goals. The observed geographical, racial and ethnic, age, sex, and temporal variations require constant monitoring and research. Funding International Agency for Research on Cancer and the Union for International Cancer Control

Geographical variability in survival of European children with central nervous system tumours

Survival for childhood central nervous system (CNS) tumours varies across Europe, partly because of the difficulty of distinguishing malignant from non-malignant disease. This study examines bias in CNS tumours survival analysis to obtain the reliable and comparable survival figures. We analysed survival data for about 15,000 children (age <15) diagnosed with CNS between 2000 and 2007, from 71 population-based cancer registries in 27 countries. We selected high-quality data based on registry-specific data quality indicators and recorded observed 1-year and 5-year survival by countries and CNS entity. We provided age-adjusted survival and used a Cox model to calculate the hazard ratios (HRs) of death, adjusting by age, site and grading by country. Recording of non-malignant lesions, use of appropriate morphology codes and completeness of life status follow-up differed among registries. Five-year survival by countries varied less when non-malignant tumours were included, with rates between 79.5% and 42.8%. The HRs of dying, for registries with good data, adjusting by age and grading, were between 0.7 and 1.2; differences were similar when site (supra- and infra-tentorial) was included. Several sources of bias affect the correct definition of CNS tumours, the completeness of incidence series and the goodness of follow-up. The European Network of Cancer Registries needs to improve childhood cancer registration and stress the need to update the International Classification for Cancer. Since survival differences persisted even when restricting the analysis to registries with satisfactory data, and since diagnosis of CNS tumours is difficult and treatment complex, national plans must aim for the revision of the diagnosis and the coordination of care, with adequate national and international networks

Geographical variability in survival of European children with central nervous system tumours

Survival for childhood central nervous system (CNS) tumours varies across Europe, partly because of the difficulty of distinguishing malignant from non-malignant disease. This study examines bias in CNS tumours survival analysis to obtain the reliable and comparable survival figures. We analysed survival data for about 15,000 children (age <15) diagnosed with CNS between 2000 and 2007, from 71 population-based cancer registries in 27 countries. We selected high-quality data based on registry-specific data quality indicators and recorded observed 1-year and 5-year survival by countries and CNS entity. We provided age-adjusted survival and used a Cox model to calculate the hazard ratios (HRs) of death, adjusting by age, site and grading by country. Recording of non-malignant lesions, use of appropriate morphology codes and completeness of life status follow-up differed among registries. Five-year survival by countries varied less when non-malignant tumours were included, with rates between 79.5% and 42.8%. The HRs of dying, for registries with good data, adjusting by age and grading, were between 0.7 and 1.2; differences were similar when site (supra- and infra-tentorial) was included. Several sources of bias affect the correct definition of CNS tumours, the completeness of incidence series and the goodness of follow-up. The European Network of Cancer Registries needs to improve childhood cancer registration and stress the need to update the International Classification for Cancer. Since survival differences persisted even when restricting the analysis to registries with satisfactory data, and since diagnosis of CNS tumours is difficult and treatment complex, national plans must aim for the revision of the diagnosis and the coordination of care, with adequate national and international networks

Survival of 86,690 patients with thyroid cancer: A population-based study in 29 European countries from EUROCARE-5

Background Incidence rates of thyroid cancer (TC) increased in several countries during the last 30 years, while mortality rates remained unchanged, raising important questions for treatment and follow-up of TC patients. This study updates population-based estimates of relative survival (RS) after TC diagnosis in Europe by sex, country, age, period&nbsp;and histology. Methods Data from 87 cancer registries in 29 countries were extracted from the EUROCARE-5 dataset. One- and 5-year RS were estimated using the cohort approach for 86,690 adult TC patients diagnosed in 2000–2007 and followed-up to 12/31/2008. RS trends in 1999–2007 and 10-year RS in 2005–2007 were estimated using the period approach. Results In Europe 2000–2007, 5-year RS after TC was 88% in women and 81% in men. Survival rates varied by country and were strongly correlated (Pearson ρ&nbsp;=&nbsp;75%) with country-specific incidence rates. Five-year RS decreased with age (in women from &gt;95% at age 15–54 to 57% at age 75+), from 98% in women and 94% in men with papillary TC to 14% in women and 12% in men with anaplastic TC. Proportion of papillary TC&nbsp;varied by country and increased over time, while survival rates were similar across areas and periods. In 1999–2007, 5-year RS increased by five percentage points for all TCs&nbsp;but only by two for papillary&nbsp;and by four for follicular TC. Ten-year RS in 2005–2007 was 89% in women and 79% in men. Conclusions The reported increasing TC survival trend and differences by area are mainly explained by the varying histological case-mix of cases