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.

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

FERRETTI, Stefano;
2014

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.
2014
Stiller, C. A; Trama, A.; Brewster, D. H.; Verne, J.; Bouchardy, C.; Navarro, C.; Chirlaque, M. D.; Marcos Gragera, R.; Visser, O.; Serraino, D.; Weiderpass, E.; Dei Tos, A. P.; Ascoli, V.; Zielonk, N.; Van Eycken, E.; Sundseth, H.; Hedelin, G.; Bouvier, A. M.; Woronoff, A. S.; Buemi, A.; Tretarre, B.; Colonna, M.; Bara, S.; Ganry, O.; Grosclaude, P.; Baconnier, S.; Holleczek, B.; Geissler, J.; Wartenberg, M.; Tryggvadottir, L.; Deady, S.; Bellu, F.; Ferretti, Stefano; Serraino, D.; Vercelli, M.; Vitarelli, S.; Federico, M.; Fusco, M.; Michiara, M.; Giacomin, A.; Tumino, R.; Mangone, L.; Falcini, F.; Senatore, G.; Budroni, M.; Piffer, S.; Crocetti, E.; La Rosa, F.; Tagliabue, G.; Zambon, P.; Gatta, G.; Gronchi, A.; Licitra, L.; Ruzza, M.; Sowe, S.; Capocaccia, R.; De Angelis, R.; Mallone, S.; Tavilla, A.; Dei Tos, A. P.; England, K.; Ursin, G.; Rachtan, J.; Gozdz, S.; Zwierko, M.; Bielska Lasota, M.; Slowinski, J.; Miranda, A.; Safaei Diba, C.; Primic Zakelj, M.; Mateos, A.; Bidaurrazaga, J.; Torrella Ramos, A.; Marcos Gragera, R.; Ardanaz, E.; Galceran, J.; Martinez Garcia, C.; Sanchez Perez, M. J.; Melchor, J. M.; Adolfsson, J.; Lambe, M.; Möller, T. R.; Ringborg, U.; Jundt, G.; Usel, M.; Ess, S. M.; Spitale, A.; Konzelmann, I.; Lutz, J. M.; Coebergh, J. W. W.; Visser, O.; Otter, R.; Siesling, S.; van der Zwan, J. M.; Schouten, H.; Greenberg, D. C.; Wilkinson, J.; Roche, M.; Verne, J.; Meechan, D.; Lawrence, G.; Mackay, J.; Gavin, A.; Brewster, D. H.; Kunkler, I.; White, C.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11392/2338623
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