Over 17,688 cases of rare thoracic cancers were selected based on the list of the RACECARE project.
Mesothelioma was the most common tumour (19 per million per year) followed by epithelial tumours of the trachea and thymus (1.3 and 1.7, respectively). The age standardised incidence rates of epithelial tumours of the trachea was double in Eastern and Southern Europe versus the other European regions: 2 per million per year. Epithelial tumours of the thymus had the lowest incidence in Northern and Eastern Europe and UK and Ireland and somewhat higher incidence in Central and Southern Europe. Highest incidence in mesothelioma was seen in UK and Ireland and lowest in Eastern Europe.
Patients with tumours of the thymus had the best prognosis (1-year survival 85%, 66%at 5 years). Five year survival was lowest for the mesothelioma 5%compared to 14%of patients with tumours of the trachea. Mesothelioma was the most prevalent rare cancer (12,000 cases), followed by thymus (7000) and trachea (1400).
Cancer Registry (CR) data play an important role in revealing the burden of rare thoracic cancers and monitoring the effect of regulations on asbestos use and smoking related policies.