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Eur J Surg Oncol. 2019 Jan;45(1):67-74. doi: 10.1016/j.ejso.2017.09.025. Epub 2017 Nov 3.

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

Collaborators (118)

Hackl M18, Van Eycken E19, Henau K19, Dimitrova N20, Sekerija M21, Dušek L22, Mägi M23, Malila N24, Leinonen M24, Velten M25, Troussard X26, Bouvier V27, Guizard AV28, Bouvier AM29, Arveux P30, Maynadié M31, Woronoff AS32, Robaszkiewicz M33, Baldi I34, Monnereau A35, Tretarre B36, Colonna M36, Molinié F37, Bara S38, Schvartz C39, Lapôtre-Ledoux B40, Grosclaude P41, Stabenow R42, Luttmann S43, Nennecke A44, Engel J45, Schubert-Fritschle G46, Heidrich J47, Holleczek B48, Jónasson JG49, Clough-Gorr K50, Comber H50, Mazzoleni G51, Giacomin A52, Sardo AS53, Barchielli A54, Serraino D55, De Angelis R56, Mallone S56, Tavilla A56, Pierannunzio D56, Rossi S56, Santaquilani M56, Knijn A56, Pannozzo F57, Gennaro V58, Benfatto L58, Ricci P59, Autelitano M60, Spagnoli G61, Fusco M62, Usala M63, Vitale F64, Michiara M65, Tumino R66, Mangone L67, Falcini F68, Ferretti S68, Filiberti RA69, Marani E70, Iannelli A71, Sensi F72, Piffer S73, Gentilini M73, Madeddu A74, Ziino A75, Maspero S76, Candela P77, Stracci F78, Tagliabue G79, Rugge M80, Trama A81, Gatta G81, Botta L81, Capocaccia R81, Pildava S82, Smailyte G83, Calleja N84, Johannesen TB85, Rachtan J86, Góźdź S87, Błaszczyk J88, Kępska K88, de Lacerda GF89, José Bento M90, Miranda A91, Safaei Diba C91, Almar E92, Larrañaga N93, de Munain AL93, Torrella-Ramos A94, Díaz García JM95, Marcos-Gragera R96, Josè Sanchez M97, Navarro C98, Salmeron D98, Moreno-Iribas C99, Galceran J100, Carulla M101, Mousavi M102, Bouchardy C103, Ess SM104, Bordoni A105, Konzelmann I106, Rashbass J107, Gavin A108, Brewster DH108, Huws DW109, Visser O110, Bielska-Lasota M111, Primic-Zakelj M112, Kunkler I113, Benhamou E114.

Author information

1
Dpt of Medical Oncology, Centre Leon Berard, University Claude Bernard LyonI, Lyon, France. Electronic address: isabelle.ray-coquard@lyon.unicancer.fr.
2
AnnaLisa Trama, Fondazione IRCCS istituto nazionale dei tumori Milan, Italy.
3
Charing Cross Hospital, Campus of Imperial College London, Fulham Palace Rd, W68RF London, UK.
4
Dept of Surgery and Cancer, Imperial College London, UK.
5
Medical Oncology, Dpt Gustave Roussy Institution, Villejuif, France.
6
Medical Oncology, Department of Urology and Gynecology, Istituto Nazionale Tumori - IRCSS - Fondazione G. Pascale, Naples Italy.
7
Dept of Gynecologic Oncology, Institute for Cancer Genetics and Informatics, Oslo University Hospital, Oslo, Norway.
8
Department of Obstetrics and Gynecology, San Raffaele Scientific Institute, Milan, Italy.
9
Department of Women's and Children's Health, Division of Obstetrics and Gynecology, Karolinska Institutet/University Hospital, 171 76 Stockholm, Sweden.
10
Department of Obstetrics and Gynaecology, Radboudumc, PO Box 9101, 6500 HB Nijmegen, The Netherlands.
11
Department of Gynecology with Center for Oncological Surgery, European Competence Center for Ovarian Cancer, Campus Virchow Klinikum, Medical University of Berlin, Germany.
12
AP-HP, Université Paris Descartes, France.
13
Gynecologic Oncology Unit, Fondazione IRCCS istituto nazionale dei tumori Milan, Italy.
14
Center Gynaecologic Oncology Amsterdam (CGOA), Netherlands Cancer Institute, University of Amsterdam & Gynaecologic Oncology KU Leuven, The Netherlands.
15
Dept. of Gynaecological Oncology, The Norwegian Radium Hospital, Division of Cancer Medicine Oslo University Hospital, PO Box 4950 Nydalen, 0424 Oslo, Norway.
16
Gynaecological Oncologist, University Hospital Leuven, European Union, Herestraat 49, B-3000 Leuven, Belgium.
17
UCL Cancer Institute, University College London, UK.
18
Austrian National Cancer Registry, Austria.
19
Belgian Cancer Registry, Belgium.
20
Bulgaria Cancer Registry, Bulgaria.
21
Croatian Cancer Registry, Croatia.
22
Czech National Cancer Registry, Czech Republic.
23
Estonian Cancer Registry, Estonia.
24
Finnish Cancer Registry, Finland.
25
Bas Rhin Cancer Registry, France.
26
Basse Normandie Haematological Malignancies Registry, France.
27
Calvados Digestive Tract Registry, France.
28
Calvados, Registre Gèneral des Tumeurs, France.
29
Côte d'Or Digestive Tract Registry, Burgundy, France.
30
Côte d'Or Gynaecologic Cancer registry, France.
31
Côte d'Or Haematological Malignancies Cancer Registry, France.
32
Doubs Cancer Registry, France.
33
Finistère Digestive Tract Registry, France.
34
Gironde CNS Tumour Registry, France.
35
Gironde Haematological Malignancies, France.
36
Hérault Cancer Registry, France.
37
Isère Cancer Registry, France.
38
Loire-Atlantique/Vendée Cancer Registry, France.
39
Manche Cancer Registry, France.
40
Marne & Ardennes, Thyroid, France.
41
Somme Cancer Registry, France.
42
Tarn Cancer Registry, France.
43
Berlin; Brandenburg; Mecklenburg-West Pomerania; Saxony; Saxony-Anhalt; Thüringen Cancer Registry, Germany.
44
Bremen Cancer Registry, Leibniz Institute for Prevention Research and Epidemiology Hamburg Cancer Registry, Germany.
45
Hamburg Cancer Registry, Germany.
46
Munich Cancer Registry, Germany.
47
North Rhine-Westphalia Cancer Registry, Germany.
48
Saarland Cancer Registry, Germany.
49
Icelandic Cancer Registry, Germany.
50
National Cancer Registry, Ireland.
51
Alto Adige Cancer Registry, Italy.
52
Biella Cancer Registry, Italy.
53
Catanzaro Cancer Registry, Italy.
54
Firenze-Prato Cancer Registry, Italy.
55
Friuli Venezia Giulia, CRO Aviano National Cancer Institute, Italy.
56
Istituto Superiore di Sanità, Rome, Italy.
57
Latina Cancer Registry, Italy.
58
Liguria Mesothelioma Registry, Italy.
59
Mantova Cancer Registry, Italy.
60
Milano Cancer Registry, Italy.
61
Modena Cancer Registry, Italy.
62
Napoli 3 South Cancer Registry, Italy.
63
Nuoro Cancer Registry, Italy.
64
Palermo Cancer Registry, Italy.
65
Parma Cancer Registry, Italy.
66
Ragusa Cancer Registry, Italy.
67
Reggio Emilia Cancer Registry, Italy.
68
Romagna Cancer Registry, Italy.
69
RT Ferrara Cancer Registry, Italy.
70
RTR Liguria, IRCCS AOU SM-IST, Italy.
71
Salerno Cancer Registry, Italy.
72
Sassari Cancer Registry, Italy.
73
Servizio Epidemiologia Clinica e Valutativa, Trento, Italy.
74
Siracusa Cancer Registry, Italy.
75
Sondrio Cancer Registry, Italy.
76
Trapani Cancer Registry, Italy.
77
Umbria Cancer Registry, Italy.
78
Varese Province Cancer Registry, Fondazione IRCCS Istituto Nazionale dei Tumori, Italy.
79
Veneto Cancer Registry, Italy.
80
Fondazione IRCCS, National Cancer Institute, Milan, Italy.
81
Latvian Cancer Registry, Latvia.
82
Lithuaian Cancer Registry, Lithuania.
83
Malta National Cancer Registry, Health Information and Research, Malta.
84
Norwegian Cancer Registry, Norway.
85
Cracow Cancer Registry, Poland.
86
Kielce Cancer Registry, Poland.
87
Lower Silesia (Wroclaw); Cancer Registry, Poland.
88
Açores Cancer Registry, Poland.
89
Northern Portugal Cancer Registry, Portugal.
90
Southern Portugal Cancer Registry, Portugal.
91
Slovakian National Cancer Registry, Slovakia.
92
Albacete. Castilla-La Mancha Cancer Registry, Spain.
93
Basque Country Cancer Registry, Spain.
94
Castellón-Valencia (breast); Cancer Registry, Spain.
95
Cuenca Cancer Registry, Ecuador.
96
Girona Cancer Registry, Spain.
97
Granada Cancer Registry, CIBERESP, ibs, Granada, Spain.
98
Murcia Cancer Registry, CIBERESP, IMIB-Arrixaca, Spain.
99
Navarra Cancer Registry, CIBERESP, Spain.
100
Tarragona Cancer Registry, Spain.
101
Basel Cancer Registry, Switzerland.
102
Geneva Cancer Registry, Switzerland.
103
Grisons-Glarus, St. Gallen Cancer Registry, Switzerland.
104
Ticino Cancer Registry, Switzerland.
105
Valais Cancer Registry, Switzerland.
106
Public Health England, United Kingdom.
107
Northern Ireland Cancer Registry, Ireland.
108
Scotland Cancer Registry, United Kingdom.
109
Welsh Cancer Intelligence and Surveillance Unit, United Kingdom.
110
The Netherlands Cancer Registry, The Netherlands.
111
National Institute of Public Health-NIH, Warszawa, Poland.
112
Cancer Registry of Republic of Slovenia, Slovenia.
113
Edinburgh Cancer Centre, University of Edinburgh, United Kingdom.
114
Institut de Cancérologie Gustave Roussy, Villejuif, France.

Abstract

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.

KEYWORDS:

Gynecologic cancer; Rare cancer

PMID:
29108961
DOI:
10.1016/j.ejso.2017.09.025
[Indexed for MEDLINE]

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