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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)

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 D...

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Bibliographic Details
Main Authors: Kasper, Bernd (Author) , Hohenberger, Peter (Author)
Format: Article (Journal)
Language:English
Published: 23 June 2017
In: Annals of oncology
Year: 2017, Volume: 28, Issue: 10, Pages: 2399-2408
ISSN:1569-8041
DOI:10.1093/annonc/mdx323
Online Access:Verlag, Volltext: http://dx.doi.org/10.1093/annonc/mdx323
Verlag, Volltext: https://academic-oup-com.ezproxy.medma.uni-heidelberg.de/annonc/article/28/10/2399/3884601
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Author Notes:B. Kasper, C. Baumgarten, J. Garcia, S. Bonvalot, R. Haas, F. Haller, P., Hohenberger, N. Penel, C. Messiou, W.T. van der Graaf and A. Gronchi, on behalf of the Desmoid Working Group
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Summary: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.
Item Description:Gesehen am 27.08.2018
Physical Description:Online Resource
ISSN:1569-8041
DOI:10.1093/annonc/mdx323

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