title: Management of desmoid tumours: A nationwide survey of labelled reference centre networks in France.
abstract: OBJECTIVE
  The optimal management of rare tumours (i.e. from accurate diagnosis to management in reference centres) is a public health challenge. In 2009, the French National Cancer Institute (INCa) identified and financially supported the two expert networks for pathological and clinical diagnosis and management of soft tissue tumours.
  
  
  METHODS
  The activities of both networks were prospectively collected using a nationwide database (rreps.org). Data describing the diagnosis management of 863 successive cases of desmoids tumours (DT) were prospectively collected from 2010 to 2013 and analysed.
  
  
  RESULTS
  The number of confirmed DT constantly improved from January 2010 to December 2013 (from 173 to 273 cases per year); the expected incidence ranged from 132 to 330 cases/year. The rate of cases diagnosed with core-needle biopsies and CTNNB1 mutational status analysis increased from 30.6 to 40.7% and from 87.8 to 94.1%, respectively. The mean delay for pathological diagnosis confirmation constantly decreased from 107 to 47 d. Among the 846 adult patients, 414 (48.9%) patients were treated by reference centres. The rate of patients managed by reference centres constantly increased with time from 36.9 to 49.5% since 2010. The median management time of the referral centres constantly decreased from 440 to 67 d.
  
  
  CONCLUSIONS
  The two expert networks worked synergistically and improved diagnosis modalities of rare desmoid tumours at a national level. The impact of management by expert networks on the outcome will be prospectively analysed in the future.
authors.affiliation: Medical Oncology Department, Centre Oscar Lambret, 3 rue Combemale, 59020 Lille Cedex, France; SIRIC OncoLille Consortium, Clinical Research and Methodology Platform, 59037 Lille, France. Electronic address: n-penel@o-lambret.fr.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Penel
authors.affiliation: Pathology Department, Institut Bergonié, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: j.coindre@bordeaux.unicancer.fr.
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Coindre
authors.affiliation: Surgery Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France; Surgery Department, Institut Curie, 26 Rue d'Ulm, 75005 Paris, France. Electronic address: sylvie.bonvalot@curie.fr.
authors.forename: Sylvie
authors.initials: S
authors.lastname: Bonvalot
authors.affiliation: Medical Oncology Department, Institut Bergonie, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: a.italiano@bordeaux.unicancer.fr.
authors.forename: Antoine
authors.initials: A
authors.lastname: Italiano
authors.affiliation: Pathology Department, Institut Bergonié, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: a.neuville@bordeaux.unicancer.fr.
authors.forename: Agnès
authors.initials: A
authors.lastname: Neuville
authors.affiliation: Medical Oncology Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Axel.LECESNE@gustaveroussy.fr.
authors.forename: Axel
authors.initials: A
authors.lastname: Le Cesne
authors.affiliation: Pathology Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Philippe.TERRIER@gustaveroussy.fr.
authors.forename: Philippe
authors.initials: P
authors.lastname: Terrier
authors.affiliation: Medical Oncology Department, Centre Léon Bérard, 28 rue Laennec, 69008 Lyon, France. Electronic address: isabelle.ray-coquard@lyon.unicancer.fr.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Ray-Coquard
authors.affiliation: Pathology Department, Centre Léon Bérard, 28 rue Laennec, 69008 Lyon, France. Electronic address: dominique.ranchere-vince@lyon.unicancer.fr.
authors.forename: Dominique
authors.initials: D
authors.lastname: Ranchere-Vince
authors.affiliation: Pathology Department, Centre Oscar Lambret, 3 rue Combemale, 59020 Lille cedex, France. Electronic address: ym-robin@o-lambret.fr.
authors.forename: Yves-Marie
authors.initials: YM
authors.lastname: Robin
authors.affiliation: Medical Oncology Department, Centre Georges-François Leclerc, 1 Rue du Professeur Marion, 21000 Dijon, France. Electronic address: nisambert@cgfl.fr.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Isambert
authors.affiliation: Department of Surgical Oncology, Institut universitaire du cancer Toulouse Oncopole, 1 avenue Joliot-Curie, IUCT-O, 31059 Toulouse Cedex 9, France. Electronic address: Ferron.Gwenael@claudiusregaud.fr.
authors.forename: Gwennaël
authors.initials: G
authors.lastname: Ferron
authors.affiliation: Medical Oncology Department, Hôpital de la Timone, 264 Rue Saint-Pierre, 13385 Marseille, France. Electronic address: Florence.DUFFAUD@ap-hm.fr.
authors.forename: Florence
authors.initials: F
authors.lastname: Duffaud
authors.affiliation: Medical Oncology Department, Institut Paoli Calmette, 232 Boulevard de Sainte-Marguerite, 13009 Marseille, France. Electronic address: bertuccif@ipc.unicancer.fr.
authors.forename: François
authors.initials: F
authors.lastname: Bertucci
authors.affiliation: Medical Oncology Department, Centre Alexis Vautrin, 6 Avenue de Bourgogne, 54500 Nancy, France. Electronic address: m.rios@nancy.unicancer.fr.
authors.forename: Maria
authors.initials: M
authors.lastname: Rios
authors.affiliation: Surgery Department, Institut Bergonie, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: e.stoeckle@bordeaux.unicancer.fr.
authors.forename: Eberhad
authors.initials: E
authors.lastname: Stoeckle
authors.affiliation: Radiotherapy Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Cecile.LEPECHOUX@gustaveroussy.fr.
authors.forename: Cécile
authors.initials: C
authors.lastname: Le Pechoux
authors.affiliation: Medical Oncology Department, Centre Henri Becquerel, Rue d'Amiens, 76000 Rouen, France. Electronic address: cecile.guillemet@chb.unicancer.fr.
authors.forename: Cécile
authors.initials: C
authors.lastname: Guillemet
authors.affiliation: RRePs and NetSarc networks, Institut Bergonié, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: J.Courreges@bordeaux.unicancer.fr.
authors.forename: Jean-Baptiste
authors.initials: JB
authors.lastname: Courreges
authors.affiliation: Medical Oncology Department, Centre Léon Bérard, 28 rue Laennec, 69008 Lyon, France. Electronic address: jean-yves.blay@lyon.unicancer.fr.
authors.forename: Jean-Yves
authors.initials: JY
authors.lastname: Blay
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000235:genetics; D062005:Biopsy, Large-Core Needle; D002648:Child; D003299:Cooperative Behavior; D004252:DNA Mutational Analysis; D016208:Databases, Factual; D019033:Delivery of Health Care, Integrated / Q000458:organization & administration / Q000592:standards; D055088:Early Detection of Cancer; D005260:Female; D018222:Desmoid Tumors / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005602:France / Q000453:epidemiology; D020022:Genetic Predisposition to Disease; D019538:Health Care Surveys; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009154:Mutation; D010348:Patient Care Team; D010641:Phenotype; D011237:Predictive Value of Tests; D015397:Program Evaluation; D058996:Quality Improvement; D019984:Quality Indicators, Health Care; D012017:Referral and Consultation; D013997:Time Factors; D061665:Time-to-Treatment; D016896:Treatment Outcome; D055815:Young Adult; D051176:beta Catenin / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; C495270:CTNNB1 protein, human; D051176:beta Catenin
keywords: Desmoid tumours; Expert network; Key indicators of activity; Rare tumours; Reference centres