TY - JOUR
T1 - Rare ovarian tumours: Epidemiology, treatment challenges in and outside a network setting
AU - Ray-Coquard, I.
AU - Trama, Annalisa
AU - Seckl, M. J.
AU - Fotopoulou, C.
AU - Pautier, P.
AU - Pignata, S.
AU - Kristensen, G.
AU - Mangili, G.
AU - Falconer, H.
AU - Massuger, L.
AU - Sehouli, J.
AU - Pujade-Lauraine, E.
AU - Lorusso, D.
AU - Amant, F.
AU - Rokkones, E.
AU - Vergote, I.
AU - Ledermann, J. A.
AU - AUTHOR GROUP
AU - Hackl, Monika
AU - van Eycken, Elizabeth
AU - Henau, Kris
AU - Dimitrova, Nadya
AU - Sekerija, Mario
AU - Dušek, Ladislav
AU - Mägi, Margit
AU - Malila, Nea
AU - Leinonen, Maarit
AU - Velten, Michel
AU - Troussard, Xavier
AU - Bouvier, Veronique
AU - Guizard, Anne-Valérie
AU - Bouvier, Anne-Marie
AU - Arveux, Patrick
AU - Maynadié, Marc
AU - Woronoff, Anne-Sophie
AU - Robaszkiewicz, Michel
AU - Baldi, Isabelle
AU - Monnereau, Alain
AU - Tretarre, Brigitte
AU - Colonna, Marc
AU - Molinié, Florence
AU - Bara, Simona
AU - Schvartz, Claire
AU - Lapôtre-Ledoux, Bénédicte
AU - Grosclaude, Pascale
AU - Stabenow, Roland
AU - Luttmann, Sabine
AU - Nennecke, Alice
AU - Engel, Jutta
AU - Schubert-Fritschle, Gabriele
AU - Heidrich, Jan
PY - 2019/1/1
Y1 - 2019/1/1
N2 - More than 50% of all gynaecological cancers can be classified as rare tumours (defined as an annual incidence of <6 per 100,000) and such tumours represent an important challenge for clinicians. Rare cancers account for more than one fifth of all new cancer diagnoses, more than any of the single common cancers alone. Reviewing the RARECAREnet database, some of the tumours occur infrequently, whilst others because of their natural history have a high prevalence, and therefore appear to be more common, although their incidence is also rare. Harmonization of medical practice, guidelines and novel trials are needed to identify rare tumours and facilitate the development of new treatments. Ovarian tumours are the focus of this review, but we comment on other rare gynaecological tumours, as the diagnosis and treatment challenges faced are similar. This requires European collaboration, international partnerships, harmonization of treatment and collaboration to overcome the regulatory barriers to conduct international trials. Whilst randomized trials can be done in many tumour types, there are some for which conducting even single arm studies may be challenging. For these tumours alternative study designs, robust collection of data through national registries and audits could lead to improvements in the treatment of rare tumours. In addition, concentring the care of patients with rare tumours into a limited number of centres will help to build expertise, facilitate trials and improve outcomes
AB - More than 50% of all gynaecological cancers can be classified as rare tumours (defined as an annual incidence of <6 per 100,000) and such tumours represent an important challenge for clinicians. Rare cancers account for more than one fifth of all new cancer diagnoses, more than any of the single common cancers alone. Reviewing the RARECAREnet database, some of the tumours occur infrequently, whilst others because of their natural history have a high prevalence, and therefore appear to be more common, although their incidence is also rare. Harmonization of medical practice, guidelines and novel trials are needed to identify rare tumours and facilitate the development of new treatments. Ovarian tumours are the focus of this review, but we comment on other rare gynaecological tumours, as the diagnosis and treatment challenges faced are similar. This requires European collaboration, international partnerships, harmonization of treatment and collaboration to overcome the regulatory barriers to conduct international trials. Whilst randomized trials can be done in many tumour types, there are some for which conducting even single arm studies may be challenging. For these tumours alternative study designs, robust collection of data through national registries and audits could lead to improvements in the treatment of rare tumours. In addition, concentring the care of patients with rare tumours into a limited number of centres will help to build expertise, facilitate trials and improve outcomes
UR - http://www.scopus.com/inward/record.url?scp=85032890143&partnerID=8YFLogxK
U2 - https://doi.org/10.1016/j.ejso.2017.09.025
DO - https://doi.org/10.1016/j.ejso.2017.09.025
M3 - Article
C2 - 29108961
SN - 0748-7983
VL - 45
SP - 67
EP - 74
JO - European Journal of Surgical Oncology
JF - European Journal of Surgical Oncology
IS - 1
ER -