Information network on rare cancers Work Package 4 Information on epidemiology of rare cancers Riccardo Capocaccia and Sandra Mallone Cancer Epidemiology Unit Istituto Superiore di Sanità, Rome - Italy

Information network on rare cancers Work Package 4: Epidemiology of rare cancers Partners involved Associated: INT, UEDIN, IKNL, ECPC, NOH, NCR, CSF, OILJ Collaborating: RCE, EPAAC, ESO, INSERM, EUROCARE, ESMO, LeukaNet, ECCO

Information network on rare cancers Objectives To revise the list of rare cancers To collect and disseminate information on: - updated epidemiological indicators - the health care pathways for rare cancers Deliverable (No.7) Report on the health care pathway study and on the updated epidemiological indicators WP 4 Epidemiology of rare cancers

Information network on rare cancers Milestones Consensus meeting to revise list of rare cancers Partners meeting to discuss quality check and analysis necessary to update epidemiological indicators and estimate trends Meeting with CRs, together with WP5, to discuss health care pathway study WP 4 Epidemiology of rare cancers

Information network on rare cancers List of rare cancer The list of rare cancers which derives from the ICD-O-3 (186 identified rare cancers - around 4 million people in the European Union affected by rare cancers) will be revised and updated (refinements suggested by previous analyses, new entities) A consensus meeting is envisioned to convene the group of international experts and oncologic societies to revise the list. A contact will be established with NCI rare cancers study group: which level of coordination WP 4 Epidemiology of rare cancers

Information network on rare cancers Work Programme 7-8 / 2012: definition of the working group (basically the RARECARE wg) 9 / 2012: circulating the present list and proposed amendments 9-11 / 2012: discussion by or forum on RARECARE website 11 /2012: meeting for final delivery 12/ 2012: possibility for a second meeting, if necessary 1 /2013: new list available for endorsement from scientific associations and for analyses WP 4 Epidemiology of rare cancers List of rare cancers

Information network on rare cancers Cancer registries and data RARECARENet project will analyze incidence and follow-up data on cancer cases diagnosed in and followed- up until 2008 from more than 100 population-based cancer registries (CR) in 30 European Countries*. This activity will be carried out in collaboration with the EUROCARE project and all contributing European CRs, after seeking permission to use the EUROCARE-5 database. *Bulgaria, Croatia, Estonia, Hungary, Latvia, Lithuania, Slovakia (new joining countries). WP 4 Epidemiology of rare cancers Updated epidemiological indicators

Information network on rare cancers Analysis -1 Incidence rates will be provided for malignant cancers including cases diagnosed between 2000 and 2007, by scancer entity, sex, and country (and geographical area). Other macro-indicators: GDP level, TNEH level ? Joint Point method will be used for incidence trend analysis in the period 1990 to WP 4 Epidemiology of rare cancers Updated epidemiological indicators

Information network on rare cancers Analysis -2 Absolute and relative survival of patients diagnosed between 2000 and 2007 (i.e. patients diagnosed 5,10 or more years ago) will be provided according to the Ederer II method. Three possible different approaches: Cohort , follow-up to 2008 Period Complete Estimates will be given by cancer entity, sex, and country (and geographical area) WP 4 Epidemiology of rare cancers Updated epidemiological indicators

Information network on rare cancers Analysis -3 Survival time trends for consecutive triennia (1990 – 2007) will be estimated by using multiple regression models* to assess the mutual effect of age, sex, country ( and geographic area) and possibly stage, on risk of death and to obtain relative excess risk (RER) for categories of the same variable. RER quantify the extent to which the hazard of death in a given category differs from the hazard in the reference category, after taking into account the background risk of death in the general population. *Hakulinen and Dickman et al. (Hakulinen 1982, Dickman et al., 2004). WP 4 Epidemiology of rare cancers Updated epidemiological indicators

Information network on rare cancers Analysis -4 Estimates of observed prevalence, i.e the observed number or proportion of cancer survivors per 100,000 population will be obtained form CRs incidence and follow-up data at the index date of 31 December 2008, by using the counting method. Prevalence proportions will be disaggregated by cancer entity, age, sex and time since diagnosis. The completeness index method will be used to estimate the number of prevalent cases who were diagnosed before the start of cancer registration. WP 4 Epidemiology of rare cancers Updated epidemiological indicators

Information network on rare cancers Cancer registries and data A selection of cancer registries will send also information on the hospital of diagnosis/treatment of cancer cases. Participant national registries: Finland, Ireland, Bulgaria, Slovenia and Netherlands. Other registries can join Confidentiality problems regarding both patients and individual hospitals will be considered by shaping the published output according to the local legislation. WP 4 Epidemiology of rare cancers Health care pathways for rare cancers

Information network on rare cancers Issues 1. In which hospitals are rare cancers most frequently diagnosed/treated ? 2. Is there an association between hospital case volumes and (stage-adjusted) survival ? Is there an impact of the differing socio economical and organizational contexts on survival across countries ? WP 4 Epidemiology of rare cancers Health care pathways for rare cancers

Information network on rare cancers WP 4 Epidemiology of rare cancers Health care pathways for rare cancers Definition of Centers of Expertise Patients data Hospitals data Volumes, Outcome

Information network on rare cancers Data to be collected Patient characteristics: age at diagnosis, sex, date of diagnosis; Tumour characteristics: stage at diagnosis, tumour size, recurrencies, comorbidity; Treatment (radioterapy, chemotherapy, surgery); Date of treatment(s); Case volume defined as the number of diagnosis/operations performed per year; Second opinion, re-operation, free margins, multidisciplinary teams etc. WP 4 Epidemiology of rare cancers High Resolution studies on rare cancers

Information network on rare cancers Analysis Relative Survival will be estimated by using multiple regression models* to assess the mutual effect of the previous variables on risk of death and to obtain relative excess risk (RER) for categories of the same variable. WP 4 Epidemiology of rare cancers High Resolution studies on rare cancers

Information network on rare cancers WP 4 Epidemiology of rare cancers Timetable