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)

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, Desmoid Working Group, S Bauer, J Y Blay, F van Coevorden, P Dileo, H R Dürr, M Fiore, V Grünwald, R Jones, I Judson, C Kettelhack, K Kopeckova, A Lazar, L H Lindner, J Martin-Broto, P Rutkowski, S Stacchiotti, E Stoeckle, C Valverde, K Verhoef, E Wardelmann, M Wartenberg, 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, Desmoid Working Group, S Bauer, J Y Blay, F van Coevorden, P Dileo, H R Dürr, M Fiore, V Grünwald, R Jones, I Judson, C Kettelhack, K Kopeckova, A Lazar, L H Lindner, J Martin-Broto, P Rutkowski, S Stacchiotti, E Stoeckle, C Valverde, K Verhoef, E Wardelmann, M Wartenberg

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: EORTC/STBSG; SPAEN; aggressive fibromatosis; desmoid; patient advocacy groups; treatment algorithm.

© The Author 2017. Published by Oxford University Press on behalf of the European Society for Medical Oncology.

Figures

Figure 1.
Figure 1.
(A) Macroscopic picture of DF. Note finger-like extensions (arrow) into muscle (M). (B) Microscopic picture of DF arising from deep fascia (F). Note the infiltrative growth into skeletal muscle (arrows). (C) Screen-shot of next-generation sequencing analysis of β-catenin T41A mutation, with missense mutation A > G in only a subset of the reads.
Figure 2.
Figure 2.
Immunohistochemistry of a DF with characteristic β-catenin staining.
Figure 3.
Figure 3.
Examples of spontaneous regression of DF at different sites. (A) Intra-abdominal DF. (B) Scapular girdle DF.
Figure 4.
Figure 4.
Consensus algorithm.

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