Rare ovarian tumours: Epidemiology, treatment challenges in and outside a network setting.

Autor: Ray-Coquard I; Dpt of Medical Oncology, Centre Leon Berard, University Claude Bernard LyonI, Lyon, France. Electronic address: isabelle.ray-coquard@lyon.unicancer.fr., Trama A; AnnaLisa Trama, Fondazione IRCCS istituto nazionale dei tumori Milan, Italy., Seckl MJ; Charing Cross Hospital, Campus of Imperial College London, Fulham Palace Rd, W68RF London, UK., Fotopoulou C; Dept of Surgery and Cancer, Imperial College London, UK., Pautier P; Medical Oncology, Dpt Gustave Roussy Institution, Villejuif, France., Pignata S; Medical Oncology, Department of Urology and Gynecology, Istituto Nazionale Tumori - IRCSS - Fondazione G. Pascale, Naples Italy., Kristensen G; Dept of Gynecologic Oncology, Institute for Cancer Genetics and Informatics, Oslo University Hospital, Oslo, Norway., Mangili G; Department of Obstetrics and Gynecology, San Raffaele Scientific Institute, Milan, Italy., Falconer H; Department of Women's and Children's Health, Division of Obstetrics and Gynecology, Karolinska Institutet/University Hospital, 171 76 Stockholm, Sweden., Massuger L; Department of Obstetrics and Gynaecology, Radboudumc, PO Box 9101, 6500 HB Nijmegen, The Netherlands., Sehouli J; Department of Gynecology with Center for Oncological Surgery, European Competence Center for Ovarian Cancer, Campus Virchow Klinikum, Medical University of Berlin, Germany., Pujade-Lauraine E; AP-HP, Université Paris Descartes, France., Lorusso D; Gynecologic Oncology Unit, Fondazione IRCCS istituto nazionale dei tumori Milan, Italy., Amant F; Center Gynaecologic Oncology Amsterdam (CGOA), Netherlands Cancer Institute, University of Amsterdam & Gynaecologic Oncology KU Leuven, The Netherlands., Rokkones E; Dept. of Gynaecological Oncology, The Norwegian Radium Hospital, Division of Cancer Medicine Oslo University Hospital, PO Box 4950 Nydalen, 0424 Oslo, Norway., Vergote I; Gynaecological Oncologist, University Hospital Leuven, European Union, Herestraat 49, B-3000 Leuven, Belgium., Ledermann JA; UCL Cancer Institute, University College London, UK.
Jazyk: angličtina
Zdroj: European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology [Eur J Surg Oncol] 2019 Jan; Vol. 45 (1), pp. 67-74. Date of Electronic Publication: 2017 Nov 03.
DOI: 10.1016/j.ejso.2017.09.025
Abstrakt: Purpose of the Review: 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.
Recent Findings: 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.
Future: 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.
(Copyright © 2017 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)
Databáze: MEDLINE