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)

Bernd Kasper; C. Baumgarten; J. Garcia; Sylvie Bonvalot; R. Haas; F. Haller; P. Hohenberger; Nicolas Penel; C. Messiou; W. T. van der Graaf; A. Gronchi

doi:10.1093/annonc/mdx323

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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)

Bernd Kasper ¹ C. Baumgarten J. Garcia Sylvie Bonvalot ² R. Haas ³ F. Haller ⁴ P. Hohenberger ¹ Nicolas Penel ⁵ C. Messiou W. T. van der Graaf ⁶ A. Gronchi ⁷

1 University of Heidelberg, Medical Faculty

2 Institut Curie [Paris]

3 Antoni van Leeuwenhoek Hospital

4 FAU - Friedrich-Alexander Universität Erlangen-Nürnberg

5 CERIM - Santé publique : épidémiologie et qualité des soins-EA 2694

6 The institute of cancer research [London]

7 Molecular Targeting Unit, Department of Research [Fondazione IRCCS Istituto Nazionale dei Tumori, Milan]

Abstract : Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals' AND patients' expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet. In this paper, we focus on new findings regarding the prognostic value of mutational analysis in desmoid-type fibromatosis patients and new systemic treatment options.

Keywords : Mesh:Fibromatosis Mesh:Aggressive/therapy* Mesh:Aggressive/genetics Mesh:Aggressive/diagnosis* Mesh:Humans aggressive fibromatosis EORTC/STBSG patient advocacy groups SPAEN treatment algorithm desmoid Mesh:Randomized Controlled Trials as Topic

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Bernd Kasper, C. Baumgarten, J. Garcia, Sylvie Bonvalot, R. Haas, et al.. 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). Annals of Oncology, Elsevier, 2017, Annals of oncology official journal of the European Society for Medical Oncology, 28, pp.2399-2408. ⟨10.1093/annonc/mdx323⟩. ⟨hal-02639170⟩

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)

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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)

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