1. South West Public Health Observatory Results of an investigation into public concerns about Osteosarcoma in young people in the Helston area Dr Julia Verne Director South West Cancer Intelligence Service and Public Health Observatory

2. South West Public Health Observatory Our responsibilities  Well-being of the young people with osteosarcoma and their families –Appreciate distress of families –Constant public/private reminders –Threat to privacy –Media interest –Worry “could I have done something different to prevent my child getting this horrible disease”  The local population and their concerns –Important not to stigmatise a community

3. South West Public Health Observatory What is Osteosarcoma?  Bone cancer  One in five of all bone cancers  Commonest in adolescents and young adults but also occurs in the elderly  50% of osteosarcomas develop in the legs  25% in the shoulder  In young 60-80% develop around the knee (Huvos)

4. South West Public Health Observatory Is Osteosarcoma common?  No it is rare –In England and Wales –~450 new cases per year all ages –~100 new cases under 20 years old –~290 deaths per year all ages –~40 deaths in under 20 years old  Osteosarcoma accounts for 5% of all childrens’ cancers  Cancers in children are not common ~ 100 cases in under 16s per year in the South West  On average in a population of ~ 5 million in South West there are 7 cases per year (range 2-13) in 0-29 yr olds

5. South West Public Health Observatory What causes Osteosarcoma?  Mostly unknown –Genetics Germline mutations retinoblastoma and p53 genes (~6%) Retinoblastoma and Li-Fraumeni Syndromes (~1%) –Previous radiotherapy especially in children –In elderly a previous history of Paget’s disease and osteomyelitis –Exposure to high doses of radioactivity e.g. World War 1 factory girls and radium and mesothorium –Exposure to environmental radon does not appear to be a risk factor

6. South West Public Health Observatory Signs, symptoms and diagnosis  Diagnosis can be difficult in young people because symptoms are so commonly found in a healthy population  Pain and swelling are commonest symptoms and signs. Pain can be worse at night.  Diagnosis is by X-ray and biopsy, often other tests e.g. MRI and bone scanning are used as well

7. South West Public Health Observatory Treatment and survival  Treatment –Surgery –Radiotherapy –Chemotherapy  Survival –10-20% of patients have advanced disease at diagnosis –EUROCARE 52% 1978-1989, 60% 1985-1989 –Overall survival UK CCSG 1995-99 - 58% –South West survival 1996-2001 - 63% (+/- 17% SE)

8. South West Public Health Observatory Background to the investigation  Concern from parents about the number of cases which appeared to be from the Helston area  Media interest  Director of Public Health of West of Cornwall Primary Care Trust requested South West Cancer Intelligence Service to conduct an independent investigation

9. South West Public Health Observatory Role of Cancer Registries  Collect data on all new cases of cancer diagnosed within the region –More than one million records on database –Over 25,000 new cases of cancer registered every year  Check the accuracy of the information by comparing different sources  Monitor patterns of cancer especially over time and across geographical areas  Early warning of possible public health problems highlighting areas for action  Audit of the quality of care  Research

10. South West Public Health Observatory Prostate Cancer in Primary Care Trusts 1992-2001. Age standardised incidence rates

11. South West Public Health Observatory Prostate cancer in Primary Care Trusts. Standardised Incidence Ratio 1999-2001 (Indirect standardisation using England rates for 2000)

12. South West Public Health Observatory

13. Comparative incidence rates for melanoma

14. South West Public Health Observatory

15. All persons mortality from malignant melanoma 1998 to 2000 pooled, all ages

16. South West Public Health Observatory Patient confidentiality/ data protection  Data Protection Act  Health and Social Care Act  ONS/UKACR Guidance on release/publication of data to protect patient confidentiality –Not when denominator <1,000 –Not when number of cases <5

17. South West Public Health Observatory Patient confidentiality  Discussions held at the highest levels on presenting and publishing the results of the study  Key considerations –Families’ need for privacy –Public interest –Information already in the public domain

18. South West Public Health Observatory The investigation  The steering group –Chair Dr Julia Verne –South West Cancer Intelligence Service –Doctors involved in the care of young osteosarcoma patients: in Cornwall and at the Bristol Children’s Hospital: paediatrician and paediatric oncologists –Director of Public Health West of Cornwall PCT –Representative of Peninsula Cancer Network

19. South West Public Health Observatory Issues discussed with experts  Office for National Statistics  Bone specialist pathologist  NRPB  Child cancer epidemiologists  Sarcoma specialists

20. South West Public Health Observatory Understanding people’s concerns  Private meetings between the families at the centre of the concerns and members of the investigation team  Feedback received via Director of Public Health of other local concerns  Meeting with Mr Andrew George MP  Letters, phone calls, emails  News coverage

21. South West Public Health Observatory Key local concerns  Were there more cases in the Helston area in the past few years than one would expect by chance?  If so, was there an environmental or other cause? –Could we have protected our children –Should something be done to protect other children  Why did we not start monitoring things in the environment?  Why did we not gather more information from patients and families?  Radon, electromagnetic radiation and viruses

22. South West Public Health Observatory Outline of Study  Define question –Osteosarcoma only –Age range 0-29  Validation of cases –Real people –Duplicates –Correct diagnosis –Data checked between SWCIS/Children’s Hospital/Pathology/London/Birmingham

23. South West Public Health Observatory Outline of Study  Incidence rate and Standardised Registration Ratio for osteosarcoma –West of Cornwall PCT, Cornwall, Devon and Cornwall combined  Incidence rates for childrens’ cancers –Including radiosensitive ones

24. South West Public Health Observatory Incidence rates  Number of cancers per 100,000 –Important because Primary Care Trust populations different sizes ranging from 87,000 to 245,000  Age standardised because age is important risk factor for cancer –See Map  Standardised Registration Ratio = comparison between: – Observed (what is found) –Expected (what would be found if rate same as a standard population i.e. South West)

25. South West Public Health Observatory Percentage of population aged 19 or under (2001 Census)

26. South West Public Health Observatory Results  All cancer types 0-29 years –Slight increase in incidence rates over 20 years for the South West Region – in line with National Pattern –Nothing significant for radiosensitive tumours e.g. thyroid

27. South West Public Health Observatory Incidence of all cancers in 0-29 age group 1982-2001 Note: large year to year fluctuations for West of Cornwall PCT because of small numbers

28. South West Public Health Observatory Osteosarcoma 1983-2002 in 0-29 years  Average number of cases per year in the South West is 7 (range 2-13)  Incidence rate for South West region over 20 years is constant.  Small numbers for smaller geographical areas lead to big year to year fluctuations e.g. for Cornwall

29. South West Public Health Observatory Osteosarcoma incidence in age 0-29 1983-2002

30. South West Public Health Observatory Incidence of Osteocarcoma 1983-2002 in age 0-29 I shows how much rate could vary and still not be statistically significant. Overlap means results not statistically different

31. South West Public Health Observatory Results: Osteosarcoma 1983-2002  In 20 years there were –38 cases in Devon and Cornwall –14 cases in Cornwall –6 cases in the West of Cornwall PCT  For the 20 year period the Standardised Registration ratios (Observed to Expected) for the West of Cornwall PCT, Cornwall and Devon and Cornwall together were not significantly raised

32. South West Public Health Observatory Osteosarcoma results continued:  Between 1998-2002 there were only 4 cases diagnosed in under 30 year olds in the West of Cornwall PCT compared to the 6 originally stated to come from the Helston area.  One of the six was fictitious and one lives a long distance away.  Only two of the four have lived in Helston.  Three have attended Helston College.

33. South West Public Health Observatory Osteosarcoma results continued  The only (just) statistically significant result was for West of Cornwall PCT 1998-2002 4 cases versus ~1 expected. SRR 422 95% CI (9- 880) P=0.03  But –This can still be a chance occurrence due to chance fluctuations in very small numbers –Similar slightly increased levels are found in other PCTs across the region from time to time –If routinely monitoring this would not be a large enough increase to prompt further investigation –Recommended level for action is 20-fold increase in observed versus expected

34. South West Public Health Observatory Will we find the causes of the Osteosarcoma cases in the West of Cornwall ?  No  Statistics do not suggest a constant or recent environmental hazard  Careful history from families does not support a single environmental hazard  Small clusters of this type happen all the time all over the world by chance and the cause is very rarely found  Best described risk factors are: genetic and previous radiotherapy treatment – not risk factors in this group  Numbers of cases in Cornwall or even the South West are too small to have a chance of finding conclusive information

35. South West Public Health Observatory Will we find the causes of the Osteosarcoma cases in the West of Cornwall ?  No  Is this a disease for which there is a unique detectable previously described risk factor?  Can the effect of the risk factor on the individual be measured to identify exposure?  Can the risk factor be measured?  If the effect cannot be measured then is there an accurate way of subjects being able to quantify their exposure?

36. South West Public Health Observatory Will we find the causes of the Osteosarcoma cases in the West of Cornwall?  Radon: –South West study found no link with radon –UK-wide study of childhood cancers found no association with radon –Life-time measurement of home levels in the one life-time resident of Helston area were within acceptable limits or reduced to these  Electromagnetic radiation –No evidence of specific link from scientific literature

37. South West Public Health Observatory Will we find the causes of the Osteosarcoma cases in the West of Cornwall?  Viruses –Viruses have been proposed as possible explanations for observed clusters of leukaemia but no specific agent has been identified –Interest is increasing in similar hypotheses for other childhood cancers –Large study will be published later in 2004 on geographical distribution of childhood cancer and clustering which will indirectly look at virus hypothesis –At present there are no studies published which suggest a viral cause for osteosarcoma

38. South West Public Health Observatory Do we need further studies on Osteosarcoma?  Yes  We need to continue to monitor survival and test treatments  Care of older adolescents and young adults  We need to understand more about the causes –Need bigger numbers – national or international study –Role of Genetics

39. South West Public Health Observatory Conclusions  Slight increase in number of cases of osteosarcoma in 0-29 year olds in West of Cornwall PCT 1998-2003  This is most likely to be a random chance fluctuation due to very small numbers  Similar random chance fluctuations have occurred elsewhere in the region and will be occurring across the country  There have been no new cases diagnosed in Cornwall in 2003

40. South West Public Health Observatory Conclusions  There is no indication that there is a local environmental factor which caused all the cases  There is no cause for public concern  Monitoring of osteosarcoma rates by the SWCIS will continue as part of routine surveillance of cancer in the region  Discussions are beginning for a national study of osteosarcoma in young people

41. South West Public Health Observatory Our responsibilities  Patients and families –Acknowledge and appreciate distress of families –Thanks to the families for all the information they have given –Reassurance - no medical expert could have advised them to do anything different to protect their children against developing osteosarcoma –Respect their need for privacy

42. South West Public Health Observatory Our responsibilities  Local population –Reassurance no evidence for any cause for concern related to osteosarcoma –Focus should be on other well described threats to health which people can do something about e.g. smoking, diet, exercise, radon