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, S. Bonvalot, R. Haas, F. Haller, P. Hohenberger, N. Penel, C. Messiou, W. T. van der Graaf, Alessandro Gronchi, S. Bauer, J. Y. Blay, F. van Coevorden, P. Dileo, H. R. Dürr, V. Grünwald, R. Jones, I. Judson, C. KettelhackK. Kopeckova, A. Lazar, L. H. Lindner, J. Martin-Broto, P. Rutkowski, S. Stacchiotti, E. Stoeckle, C. Valverde, K. Verhoef, E. Wardelmann, M. Wartenberg, on behalf of the Desmoid Working Group

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish
Pages (from-to)2399-2408
Number of pages10
JournalAnnals of Oncology
Volume28
Issue number10
DOIs
Publication statusPublished - Jan 1 2017

Keywords

  • Aggressive fibromatosis
  • Desmoid
  • EORTC/STBSG
  • Patient advocacy groups
  • SPAEN
  • Treatment algorithm

ASJC Scopus subject areas

  • Hematology
  • Oncology

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