title: Rare thoracic cancers, including peritoneum mesothelioma.
abstract: Rare thoracic cancers include those of the trachea, thymus and mesothelioma (including peritoneum mesothelioma). The aim of this study was to describe the incidence, prevalence and survival of rare thoracic tumours using a large database, which includes cancer patients diagnosed from 1978 to 2002, registered in 89 population-based cancer registries (CRs) and followed-up to 31st December 2003. 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(1) and somewhat higher incidence in Central and Southern Europe.(2) Highest incidence in mesothelioma was seen in UK and Ireland(23) and lowest in Eastern Europe.(4) 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.
authors.affiliation: Department of Registry and Research, Comprehensive Cancer Centre The Netherlands, PO Box 330, 9700 AH Groningen, The Netherlands. s.siesling@iknl.nl
authors.forename: Sabine
authors.initials: S
authors.lastname: Siesling
authors.forename: Jan Maarten
authors.initials: JM
authors.lastname: van der Zwan
authors.forename: Isabel
authors.initials: I
authors.lastname: Izarzugaza
authors.forename: Jana
authors.initials: J
authors.lastname: Jaal
authors.forename: Tom
authors.initials: T
authors.lastname: Treasure
authors.forename: Roberto
authors.initials: R
authors.lastname: Foschi
authors.forename: Umberto
authors.initials: U
authors.lastname: Ricardi
authors.forename: Harry
authors.initials: H
authors.lastname: Groen
authors.forename: Andrea
authors.initials: A
authors.lastname: Tavilla
authors.forename: Eva
authors.initials: E
authors.lastname: Ardanaz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008654:Mesothelioma / Q000453:epidemiology* / Q000401:mortality; D008875:Middle Aged; D009375:Neoplasms, Glandular and Epithelial / Q000453:epidemiology; D010534:Peritoneal Neoplasms / Q000453:epidemiology; D011159:Population Surveillance; D015995:Prevalence; D035583:Rare Diseases / Q000453:epidemiology; D013953:Thymus Neoplasms / Q000453:epidemiology* / Q000401:mortality; D014134:Tracheal Neoplasms / Q000453:epidemiology* / Q000401:mortality