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The Desmoid Tumor Research Foundation

March 2016

Management of desmoid tumours: A nationwide survey of labelled reference centre networks in France.

Author Information

Agn�s Neuville,Antoine Italiano,Axel Le Cesne,C�cile Guillemet,C�cile Le Pechoux,Dominique Ranchere-Vince,Eberhad Stoeckle,Florence Duffaud,Fran�ois Bertucci,Gwenna�l Ferron,Isabelle Ray-Coquard,Jean-Baptiste Courreges,Jean-Michel Coindre,Jean-Yves Blay,Maria Rios,Nicolas Isambert,Nicolas Penel,Philippe Terrier,Sylvie Bonvalot,Yves-Marie Robin

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.,Medical Oncology Department, Centre Alexis Vautrin, 6 Avenue de Bourgogne, 54500 Nancy, France. Electronic address: m.rios@nancy.unicancer.fr.,Medical Oncology Department, Centre Georges-Fran�ois Leclerc, 1 Rue du Professeur Marion, 21000 Dijon, France. Electronic address: nisambert@cgfl.fr.,Medical Oncology Department, Centre Henri Becquerel, Rue d'Amiens, 76000 Rouen, France. Electronic address: cecile.guillemet@chb.unicancer.fr.,Medical Oncology Department, Centre L�on B�rard, 28 rue Laennec, 69008 Lyon, France. Electronic address: isabelle.ray-coquard@lyon.unicancer.fr.,Medical Oncology Department, Centre L�on B�rard, 28 rue Laennec, 69008 Lyon, France. Electronic address: jean-yves.blay@lyon.unicancer.fr.,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.,Medical Oncology Department, H�pital de la Timone, 264 Rue Saint-Pierre, 13385 Marseille, France. Electronic address: Florence.DUFFAUD@ap-hm.fr.,Medical Oncology Department, Institut Bergonie, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: a.italiano@bordeaux.unicancer.fr.,Medical Oncology Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Axel.LECESNE@gustaveroussy.fr.,Medical Oncology Department, Institut Paoli Calmette, 232 Boulevard de Sainte-Marguerite, 13009 Marseille, France. Electronic address: bertuccif@ipc.unicancer.fr.,Pathology Department, Centre L�on B�rard, 28 rue Laennec, 69008 Lyon, France. Electronic address: dominique.ranchere-vince@lyon.unicancer.fr.,Pathology Department, Centre Oscar Lambret, 3 rue Combemale, 59020 Lille cedex, France. Electronic address: ym-robin@o-lambret.fr.,Pathology Department, Institut Bergoni�, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: a.neuville@bordeaux.unicancer.fr.,Pathology Department, Institut Bergoni�, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: j.coindre@bordeaux.unicancer.fr.,Pathology Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Philippe.TERRIER@gustaveroussy.fr.,Radiotherapy Department, Institut Gustave Roussy, 114 Rue Edouard Vaillant, 94800 Villejuif, France. Electronic address: Cecile.LEPECHOUX@gustaveroussy.fr.,RRePs and NetSarc networks, Institut Bergoni�, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: J.Courreges@bordeaux.unicancer.fr.,Surgery Department, Institut Bergonie, 229 Cours de l'Argonne, 33000 Bordeaux, France. Electronic address: e.stoeckle@bordeaux.unicancer.fr.,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.

Publication

Summary

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. 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. 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. The two expert networks worked synergistically and improved diagnosis modalities of raredesmoid tumours at a national level. The impact of management by expert networks on the outcome will be prospectively analysed in the future.

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