title: Descriptive epidemiology of Kaposi sarcoma in Europe. Report from the RARECARE project.
abstract: Kaposi sarcoma (KS) is a virus-related malignancy which most frequently arises in skin, though visceral sites can also be involved. Infection with Kaposi sarcoma herpes virus (KSHV or HHV-8) is required for development of KS. Nowadays, most cases worldwide occur in persons who are immunosuppressed, usually because of HIV infection or as a result of therapy to combat rejection of a transplanted organ, but classic Kaposi sarcoma is predominantly a disease of the elderly without apparent immunosuppression. We analyzed 2667 KS incident cases diagnosed during 1995-2002 and registered by 75 population-based European cancer registries contributing to the RARECARE project. Total crude and age-standardized incidence rate was 0.3 per 100,000 per year with an estimated 1642 new cases per year in the EU27 countries. Age-standardized incidence rate was 0.8 per 100,000 in Southern Europe but below 0.3 per 100,000 in all other regions. The elevated rate in southern Europe was attributable to a combination of classic Kaposi sarcoma in some Mediterranean countries and the relatively high incidence of AIDS in several countries. Five-year relative survival for 2000-2002 by the period method was 75%. More than 10,000 persons were estimated to be alive in Europe at the beginning of 2008 with a past diagnosis of KS. The aetiological link with suppressed immunity means that many people alive following diagnosis of KS suffer comorbidity from a pre-existing condition. While KS is a rare cancer, it has a relatively good prognosis and so the number of people affected by it is quite large. Thus it provides a notable example of the importance of networking in diagnosis, therapy and research for rare cancers.
authors.affiliation: Childhood Cancer Research Group, Department of Paediatrics, University of Oxford, Oxford, UK. Electronic address: charles.stiller@new.ox.ac.uk.
authors.forename: C A
authors.initials: CA
authors.lastname: Stiller
authors.affiliation: Department of Preventive and Predictive Medicine, Fondazione IRCSS, Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Trama
authors.affiliation: Scottish Cancer Registry, Information Services Division, National Services Scotland Gyle Square, 1 South Gyle Crescent, Edinburgh EH12 9EB, Scotland, UK.
authors.forename: D H
authors.initials: DH
authors.lastname: Brewster
authors.affiliation: Public Health England Knowledge & Intelligence Team (South West), Grosvenor House, 149 Whiteladies Road, Bristol BS8 2RA, UK.
authors.forename: J
authors.initials: J
authors.lastname: Verne
authors.affiliation: Geneva Cancer Registry, IMSP - University of Geneva 55, Bd de la Cluse, CH-1205 Geneva, Switzerland.
authors.forename: C
authors.initials: C
authors.lastname: Bouchardy
authors.affiliation: Department of Epidemiology, Murcia Regional Health Authority, Ronda de Levante 11, 30008 Murcia, Spain; CIBER Epidemiología y Salud Pública (CIBERESP), Spain; Department of Health and Social Sciences, Universidad de Murcia, Murcia, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Navarro
authors.affiliation: CIBER Epidemiología y Salud Pública (CIBERESP), Spain.
authors.forename: M D
authors.initials: MD
authors.lastname: Chirlaque
authors.affiliation: Epidemiology Unit and Girona Cancer Registry, Oncology Coordination Plan, Department of Health, Autonomous Government of Catalonia and Descriptive Epidemiology, Genetics and Cancer Prevention Group [Girona Biomedical Research Institute (IdIBGi)], Catalan Institute of Oncology, Carrer del Sol, 15, 17004 Girona, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Marcos-Gragera
authors.affiliation: Comprehensive Cancer Centre the Netherlands, Registration & Research, PO Box 19.079, 3501 DB Utrecht, The Netherlands.
authors.forename: O
authors.initials: O
authors.lastname: Visser
authors.affiliation: SOC Epidemiologia e Biostatistica, Friuli Venezia Giulia Cancer Registry, IRCCS Centro di Riferimento Oncologico, Aviano, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Serraino
authors.affiliation: Department of Research, Cancer Registry of Norway, Oslo, Norway; Department of Community Medicine, Faculty of Health Sciences, University of Tromsø, The Arctic University of Norway, Tromsø, Norway; Department of Medical Epidemiology and Biostatistics, Karolinska Institutet, Stockholm, Sweden; Samfundet Folkhälsan, Helsinki, Finland.
authors.forename: E
authors.initials: E
authors.lastname: Weiderpass
authors.affiliation: Department of Oncology and Anatomic Pathology and General Hospital of Treviso, Piazza Ospedale 1, Treviso, Italy.
authors.forename: A P
authors.initials: AP
authors.lastname: Dei Tos
authors.affiliation: Dipartimento di Scienze Radiologiche, Oncologiche ed Anatomopatologiche, Università Sapienza, Viale Regina Elena 324, 00161 Roma, Italy.
authors.forename: V
authors.initials: V
authors.lastname: Ascoli
mesh_terms: D000328:Adult; D000368:Aged; D005060:Europe; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D012514:Sarcoma, Kaposi / Q000453:epidemiology* / Q000401:mortality; D016019:Survival Analysis
keywords: Europe; Incidence; Kaposi sarcoma; Population-based study; Prevalence; Rare cancers; Survival