Work Package 4

1
Work Package 4
Information on epidemiology of rare cancers
Riccardo Capocaccia and Sandra Mallone Cancer
Epidemiology Unit Istituto Superiore di Sanità,
Rome - Italy
2
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

3
WP 4 Epidemiology of 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

4
WP 4 Epidemiology of 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

5
WP 4 Epidemiology of 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

6
WP 4 Epidemiology of rare cancers List of 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 email 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

7
WP 4 Epidemiology of rare cancers Updated
epidemiological indicators

• Cancer registries and data
• RARECARENet project will analyze incidence and
  follow-up data on cancer cases diagnosed in
  1978-2007 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).

8
WP 4 Epidemiology of rare cancers Updated
epidemiological indicators

• 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 2007.

9
WP 4 Epidemiology of rare cancers Updated
epidemiological indicators

• 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 2000-2004, follow-up to 2008
• Period 2005-2007
• Complete 2000-2007
• Estimates will be given by cancer entity, sex,
  and country (and geographical area)

10
WP 4 Epidemiology of rare cancers Updated
epidemiological indicators

• 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).

11
WP 4 Epidemiology of rare cancers Updated
epidemiological indicators

• 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.

12
WP 4 Epidemiology of rare cancers Health care
pathways for 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.

13
WP 4 Epidemiology of rare cancers Health care
pathways for rare cancers

• Issues
• In which hospitals are rare cancers most
  frequently diagnosed/treated ?
• 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 ?

14
WP 4 Epidemiology of rare cancers Health care
pathways for rare cancers

• Definition of Centers of Expertise

Selected CRs
Hospitals data
Patients data
WP 5 Criteria and Info for Centers of Expertise
WP 4 Info on epidemiology
Volumes, Outcome
WP 7 List of Centers of Expertise
15
WP 4 Epidemiology of rare cancers High
Resolution studies 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.

16
WP 4 Epidemiology of rare cancers High
Resolution studies 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.

17
WP 4 Epidemiology of rare cancers Timetable