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Cancer Epidemiol. 2014 Dec;38(6):670-8. doi: 10.1016/j.canep.2014.09.009. Epub 2014 Oct 22.

Descriptive epidemiology of Kaposi sarcoma in Europe. Report from the RARECARE project.

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Childhood Cancer Research Group, Department of Paediatrics, University of Oxford, Oxford, UK. Electronic address:
Department of Preventive and Predictive Medicine, Fondazione IRCSS, Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy.
Scottish Cancer Registry, Information Services Division, National Services Scotland Gyle Square, 1 South Gyle Crescent, Edinburgh EH12 9EB, Scotland, UK.
Public Health England Knowledge & Intelligence Team (South West), Grosvenor House, 149 Whiteladies Road, Bristol BS8 2RA, UK.
Geneva Cancer Registry, IMSP - University of Geneva 55, Bd de la Cluse, CH-1205 Geneva, Switzerland.
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.
CIBER Epidemiología y Salud Pública (CIBERESP), Spain.
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.
Comprehensive Cancer Centre the Netherlands, Registration & Research, PO Box 19.079, 3501 DB Utrecht, The Netherlands.
SOC Epidemiologia e Biostatistica, Friuli Venezia Giulia Cancer Registry, IRCCS Centro di Riferimento Oncologico, Aviano, Italy.
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.
Department of Oncology and Anatomic Pathology and General Hospital of Treviso, Piazza Ospedale 1, Treviso, Italy.
Dipartimento di Scienze Radiologiche, Oncologiche ed Anatomopatologiche, Università Sapienza, Viale Regina Elena 324, 00161 Roma, Italy.


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.


Europe; Incidence; Kaposi sarcoma; Population-based study; Prevalence; Rare cancers; Survival

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