Abstract
The burden of rare tumors in Europe is still unknown and no generally accepted definition of them exist. The Surveillance of Rare Cancers in Europe project (funded by the European Commission) aimed at providing a definition of “rare cancer”, a list of cancers and rare cancer burden indicators, based on population-based cancer registry data, across Europe. An international consensus group agreed that incidence is the most appropriate indicator for measuring rare cancers frequency and set the threshold for rarity at 6/100,000/year. The list of rare cancers was based on the International Classification of Diseases for Oncology (ICD-O 3rd edition) and it was hierarchically structured in 2 layers based on various combinations of ICD-O morphology and topography codes: layer (1) families of tumors (relevant for the health care organisation) and layer (2) tumors clinically meaningful (relevant for clinical decision making and research). The burden indicators were estimated and are provided in this chapter.
References
Berrino et al (2009) Comparative cancer survival information in Europe. Euro J Cancer 45: 901–908
Capocaccia R, Colonna M, Corazziari I et al (2002) Measuring cancer prevalence in Europe: the EUROPREVAL Project. Ann Oncol 13: 831–839
De Angelis R, Francisci S, Baili P et al (2009) The EUROCARE-4 database on cancer survival in Europe: Data standardization, quality control and methods of statistical analysis. Euro J Cancer 45: 909 –930
European Parliament and Council of the European Communities (1999) Decision No 1295/1999/EC of the European Parliament and of the Council of 29 April 1999 adopting a programme of Community action on rare diseases within the framework for action in the field of public health (1999 to 2003)
European Parliament and Council of the European Communities (2000) Regulation (EC) No. 141/2000 of the European Parliament and of the Council of 16 December 1999 on orphan medicinal products. Official J Euro Commun L018, 22.01.2000: 1–5
European Society for Medical Oncology (2008) Improving rare cancer care in Europe. Recommendations on stakeholders actions and public policies. http://www.rarecancers.eu. Cited 2 November 2009
Eurordis survey on orphan drugs (2007) http://www.eurordis.org. Cited 2 November 2009
Ferguson WS, Forman EN (2002) Childhood cancer: past successes, future directions. Med Health R I 85: 17–22
Ferlay J, Bray F, Pisani P, Parkin DM (2004) GLOBOCAN 2002 Cancer Incidence, Mortality and Prevalence Worldwide IARC Cancer Base No. 5, version 2.0 IARC Press, Lyon
Gatta G, Ciccolallo L, Kunkler I et al (2006) Survival from rare cancers in adults: a population based study. Lancet Oncol 7(2): 132–140
Hakulinen T (1982) Cancer survival corrected for heterogeneity in patient withdrawal. Biometrics 38: 933–42
Ku GY, Ilson DH (2009) Role of neoadjuvant therapy for esophageal adenocarcinoma. Surg Oncol Clin N Am 18 (3): 533–546.
Percy C, Fritz A, Jack A, Shanmugarathan S, Sobin L, Parkin DM, Whelan S (2000) International Classification of Diseases for the Oncology (ICD-O), 3rd edn. World Health Organisation, Geneva
Trivers et al (2005) Demographic and lifestyle predictors of survival in patients with oesophageal or gastric cancers. Clin Gastroenterol Hepatol 3(3):225–30
UKCCCR Anal Cancer Trial Working Party and UK Co-ordinating Committee on Cancer Research. Epidermoid anal cancer (1996) Results from the UKCCCR randomised trial of radiotherapy alone versus radiotherapy, 5–fluorouracil, and mitomycin. Lancet 348: 1049–54
Verwei J, Casali PG, Zalcberg J, et al (2004) Progression-free survival in gastrointestinal stromal tumours with high-dose imatinib: randomised trial. Lancet 364: 1127–34
No Authors listed (2001) Very rare cancers – a problem neglected. Lancet Oncol 2 (4): 189
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Gatta, G., Capocaccia, R., Trama, A., Martínez-García, C., the RARECARE Working Group. (2010). The Burden of Rare Cancers in Europe. In: Posada de la Paz, M., Groft, S. (eds) Rare Diseases Epidemiology. Advances in Experimental Medicine and Biology, vol 686. Springer, Dordrecht. https://doi.org/10.1007/978-90-481-9485-8_17
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