1. The challenges of coding cancer of unknown primary A survey of registration and reporting practices in the UK, Ireland and Australia Claudia Oehler 1, Claire Vajdic 2, Nicola Cooper 1, John Symons 3 1 National Cancer Intelligence Network, Public Health England 2 University of New South Wales, Australia 3 Cancer of Unknown Primary Foundation, UK CRUK-NCIN Partnership Programme National Cancer Intelligence Network

2. Content Introduction to results of the survey on registration and reporting of Cancer of Unknown Primary (CUP): 1.Overview: what is CUP? why is it problematic? 2.The survey: what was its purpose? 3.The results: what did it tell us? 4.Conclusion: what next? 2Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

3. What is cancer of unknown primary? Cancer of unknown primary (CUP) is a diagnostic term for cancer which has spread from its initial location and where the original cancer cannot be determined with confidence. Context  9,762 cases and 10,812 deaths (UK, 2011); 16% 1-year relative survival; 57% emergencies (England, 2006-2010).  ~8 th most common incident cancer (ca.15% cancers present as metastases, 1/3 no obvious origin); ~5 th most common cancer cause of death.  2010 first clinical guideline by National Institute of Clinical Excellence (NICE) recommending diagnostic pathway and multi-disciplinary teams.  2013 first Peer Review Measures (internal validation by 133 Trusts). 3Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

4. Why is CUP problematic? Something 'unknown' is difficult to define and treat.  Clinically heterogeneous and complex presentations; diagnosis often difficult to determine; morphology and extent of cancer crucial for outcome.  Clinical uncertainty makes registration/coding of CUP difficult.  Codes used for reporting CUP vary substantially: England: mainly four ICD10 codes C77, C78, C79 (lymphatic, respiratory/abdominal and 'other' metastases) and C80 (primary cancer site unknown) - regional variations. Others: solely C80, or additional ICD10 codes for ill-defined primary cancer sites, such as C26, C39 or C76 (ill-defined digestive, respiratory/intrathoracic, or generic sites, e.g. 'head and neck').  Obscures accurate assessment of CUP burden; nationally & internationally. Clear definition and consistent recording of CUP crucial for accurate statistics and clinically meaningful analyses. 4Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

5. Purpose of the survey? The aim was to compare CUP registration and reporting practices in the UK, Ireland and Australia, with a view to supporting improved national/international standardisation. Methodology:  20 registration services participated: 8 in Australia, the 8 regional NCRS in England, 1 each in Wales, Scotland, Northern Ireland and Ireland.  2-part questionnaire: Section 1 - Registration: coding guidance and system; coding scenarios; death certificate only; clarification process. Section 2 - Reporting: codes used for reporting; incidence by source of diagnosis and morphology. 5Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

6. What did the survey tell us? Caveat: these are draft results; registration services will be given the opportunity to comment before publication.  To preserve the anonymity of individual responses, these are grouped: Registration offices in England and Australia are grouped as that; when individual answers are shown, these are numbered E1-E8 and A1-A8; these numbers were randomly assigned. Countries with a single national registry - Scotland, Wales, Northern Ireland and Ireland - are grouped here to obscure the identity of any individual registry until permission is obtained to attribute results by nation. When individual responses are shown, these countries are numbered X1-X4; these numbers were randomly assigned and do not correspond to the order in which they are listed.  Limitations: registration systems vary across countries; some of the differences may be due to a different casemix; a few questions were left blank / answers were unclear; April 2013, English registries merged to form National Cancer Registration Service. 6Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

7. What did the survey tell us? Survey confirmed variation in registration and reporting practices on almost every aspect covered. For example, coding guidance for CUP:  Q1_Does your registry have guidelines that specifically cover the registration of CUP? 7Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

8. What did the survey tell us? Question 4: Does a CUP cause of death get matched against a prior site-specific cancer registration? 8Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

9. What did the survey tell us? Question 11: If additional information is sought from notifier(s), who would be contacted for it? 9Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

10. What did the survey tell us? Question 18: Indicate the number of cases broken down by the basis of diagnosis/registration (source of information). 10Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

11. What did the survey tell us? Question 18: Indicate the number of cases broken down by the code used to report CUP [all translated to ICD10]. 11Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

12. What next? Beyond the survey - two key areas: registration processes and codes/coding systems. Registration processes  Guidance for CUP: clarification of notification; source of diagnosis; follow-up  Specific rules around death certificate notifications and matching to prior diagnoses (incl. clinical)  England: impact of National Cancer Registration Service merger; but what about the wider picture (UK, Ireland, Australia; Europe and beyond)? Coding systems and codes  ICDO3 codes ≠ ICD10 C-codes; better depth of data with ICDO3 (particularly metastases)? Australian method?  Impact of CUP registration definition on mortality statistics? 12Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia

13. Thanks! Thanks also go to the Australian registries for their participation. 13Coding cancer of unknown primary - a registration and reporting survey in the UK, Ireland and Australia