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
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
Information network on rare cancers
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
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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
Information network on rare cancers
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
Information network on rare cancers
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 followedup 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).
Information network on rare cancers
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.
•
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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)
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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).
Information network on rare cancers
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.
Information network on rare cancers
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.
•
Information network on rare cancers
WP 4 Epidemiology of rare cancers
Health care pathways for rare cancers
Issues
1.
2.
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 ?
Information network on rare cancers
WP 4 Epidemiology of rare cancers
Health care pathways for rare cancers
Definition of Centers of Expertise
Hospitals data
Patients data
Volumes,
Outcome
Information network on rare cancers
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.
Information network on rare cancers
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.
Information network on rare cancers
WP 4 Epidemiology of rare cancers
Timetable
2012
3
4
2013
1
2
3
2014
4
1
2
3
2015
4
1
2
Revision of the rare cancers list
Protocols for data collection
Updating epidemiological indicators
Analysis of case volumes
Analysis of High Resoluton data
Reporting
Information network on rare cancers