Surveillance of rare cancers

The widespread incidence and effects of cancer have led to a growing development in cancer prevention in the form of screening and research programs and cancer registries. Because of the low number of patients with rare cancers this improvement is not applied to the same extent to all cancer patients. This thesis has operationalized the definition of rare cancer and the proposed list of cancers as presented by the RARECARE project. We found that in Europe about half a million new patients are annually diagnosed with a rare cancer. Calculation of the complete prevalence indicated that over 4 million people who have been diagnosed with a rare type of cancer are still alive today. The five-year relative survival was lower for rare cancers (47%) than for common cancers (65%).\ud \ud More specific the burden of rare thoracic cancers (including peritoneum mesotheliomas), carcinomas of endocrine organs and rare neuroendocrine tumours in particular was estimated. We defined the burden of these rare cancers in terms of their incidence, prevalence and disease specific rates of survival. For the invasive extra-mammary Paget’s disease (EMPD) also the patients’ risk of developing secondary tumours after EMPD was determined. Of the 17,688 rare thoracic cancers, mesothelioma was the most common. In 2003 an estimated 315,000 patients with a past diagnosis of a tumours in the endocrine organs were living in the EU. The overall incidence rate for neuroendocrine tumours was 25 cases per million person-years. The highest increased risk of developing a second primary tumour was found in EMPD patients living in the South European region (SIR:2.3) or being female (SIR:1.5).\ud \ud In conclusion we recommend to establish centres of excellence for rare cancers and international networks between these centres across the EU to achieve necessary critical mass to overcome low volume related problems patients and clinicians are facing