Literature DB >> 26974708

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

Nicolas Penel1, Jean-Michel Coindre2, Sylvie Bonvalot3, Antoine Italiano4, Agnès Neuville5, Axel Le Cesne6, Philippe Terrier7, Isabelle Ray-Coquard8, Dominique Ranchere-Vince9, Yves-Marie Robin10, Nicolas Isambert11, Gwennaël Ferron12, Florence Duffaud13, François Bertucci14, Maria Rios15, Eberhad Stoeckle16, Cécile Le Pechoux17, Cécile Guillemet18, Jean-Baptiste Courreges19, Jean-Yves Blay20.   

Abstract

PURPOSE: 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.
CONCLUSION: 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.
Copyright © 2016 The Authors. Published by Elsevier Ltd.. All rights reserved.

Entities:  

Keywords:  Desmoid tumours; Expert network; Key indicators of activity; Rare tumours; Reference centres

Mesh:

Substances:

Year:  2016        PMID: 26974708     DOI: 10.1016/j.ejca.2016.02.008

Source DB:  PubMed          Journal:  Eur J Cancer        ISSN: 0959-8049            Impact factor:   9.162


  29 in total

1.  Restorative perineal anorectal amputation for a locally advanced desmoid tumour.

Authors:  Arcangelo Picciariello; M Rinaldi; M De Fazio; G Trigiante; V Papagni; V Bucaria; D F Altomare
Journal:  Int J Colorectal Dis       Date:  2020-05-01       Impact factor: 2.571

Review 2.  The Landmark Series: Desmoid.

Authors:  Marco Fiore; Aimee Crago; Rebecca Gladdy; Bernd Kasper
Journal:  Ann Surg Oncol       Date:  2021-01-01       Impact factor: 5.344

Review 3.  Intraabdominal sporadic desmoid tumors and inflammation: an updated literature review and presentation and insights on pathogenesis of synchronous sporadic mesenteric desmoid tumors occurring after surgery for necrotizing pancreatitis.

Authors:  Francesco Prete; MariaTeresa Rotelli; Alessandro Stella; Giovanna Calculli; Lucia Ilaria Sgaramella; Antonio Amati; Nicoletta Resta; Mario Testini; Angela Gurrado
Journal:  Clin Exp Med       Date:  2022-08-01       Impact factor: 5.057

4.  No Geographical Inequalities in Survival for Sarcoma Patients in France: A Reference Networks' Outcome?

Authors:  Yohan Fayet; Christine Chevreau; Gauthier Decanter; Cécile Dalban; Pierre Meeus; Sébastien Carrère; Leila Haddag-Miliani; François Le Loarer; Sylvain Causeret; Daniel Orbach; Michelle Kind; Louis-Romée Le Nail; Gwenaël Ferron; Hélène Labrosse; Loïc Chaigneau; François Bertucci; Jean-Christophe Ruzic; Valérie Le Brun Ly; Fadila Farsi; Emmanuelle Bompas; Sabine Noal; Aurore Vozy; Agnes Ducoulombier; Clément Bonnet; Sylvie Chabaud; Françoise Ducimetière; Camille Tlemsani; Mickaël Ropars; Olivier Collard; Paul Michelin; Justine Gantzer; Pascale Dubray-Longeras; Maria Rios; Pauline Soibinet; Axel Le Cesne; Florence Duffaud; Marie Karanian; François Gouin; Raphaël Tétreau; Charles Honoré; Jean-Michel Coindre; Isabelle Ray-Coquard; Sylvie Bonvalot; Jean-Yves Blay
Journal:  Cancers (Basel)       Date:  2022-05-25       Impact factor: 6.575

5.  Unraveling Desmoid-Type Fibromatosis-Specific Health-Related Quality of Life: Who Is at Risk for Poor Outcomes.

Authors:  Anne-Rose W Schut; Emma Lidington; Milea J M Timbergen; Eugenie Younger; Winette T A van der Graaf; Winan J van Houdt; Johannes J Bonenkamp; Robin L Jones; Dirk J Grünhagen; Stefan Sleijfer; Cornelis Verhoef; Spyridon Gennatas; Olga Husson
Journal:  Cancers (Basel)       Date:  2022-06-16       Impact factor: 6.575

Review 6.  Molecular Pathogenesis of Sporadic Desmoid Tumours and Its Implications for Novel Therapies: A Systematised Narrative Review.

Authors:  Thomas D McLean; Serena Duchi; Claudia Di Bella
Journal:  Target Oncol       Date:  2022-04-21       Impact factor: 4.864

Review 7.  Adult desmoid tumors: biology, management and ongoing trials.

Authors:  Nicolas Penel; Frédéric Chibon; Sébastien Salas
Journal:  Curr Opin Oncol       Date:  2017-07       Impact factor: 3.645

8.  Desmoid Fibromatosis of the Abdominal Wall: Surgical Resection and Reconstruction with Biological Matrix Egis®.

Authors:  Saveria Tropea; Simone Mocellin; Roberto Stramare; Maria Giuseppina Bonavina; Carlo Riccardo Rossi; Marco Rastrelli
Journal:  Case Rep Oncol       Date:  2017-02-17

Review 9.  Rationale for the use of tyrosine kinase inhibitors in the treatment of paediatric desmoid-type fibromatosis.

Authors:  Monika Sparber-Sauer; Daniel Orbach; Fariba Navid; Simone Hettmer; Stephen Skapek; Nadège Corradini; Michela Casanova; Aaron Weiss; Matthias Schwab; Andrea Ferrari
Journal:  Br J Cancer       Date:  2021-03-15       Impact factor: 7.640

Review 10.  An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG).

Authors:  B Kasper; C Baumgarten; J Garcia; S Bonvalot; R Haas; F Haller; P Hohenberger; N Penel; C Messiou; W T van der Graaf; A Gronchi
Journal:  Ann Oncol       Date:  2017-10-01       Impact factor: 32.976

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