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

doi:10.1093/annonc/mdx323

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)

Ann Oncol. 2017 Oct 1;28(10):2399-2408. doi: 10.1093/annonc/mdx323.

Authors

B Kasper¹, C Baumgarten², J Garcia², S Bonvalot³, R Haas⁴, F Haller⁵, P Hohenberger⁶, N Penel⁷, C Messiou⁸, W T van der Graaf⁹, A Gronchi¹⁰; Desmoid Working Group

Collaborators

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

Affiliations

¹ Sarcoma Unit, Interdisciplinary Tumor Center, Mannheim University Medical Center, University of Heidelberg, Mannheim;. Electronic address: [email protected].
² SPAEN Sarcoma PAtients EuroNet e.V, Wölfersheim, Germany.
³ Department of Surgical Oncology, Institut Curie, PSL University, Paris, France.
⁴ Department of Radiotherapy, The Netherlands Cancer Institute-Antoni van Leeuwenhoek Hospital, Amsterdam;; Department of Radiotherapy, Leiden University Medical Center, Leiden, The Netherlands.
⁵ Institute of Pathology, Friedrich Alexander University Erlangen, Erlangen, Germany.
⁶ Sarcoma Unit, Interdisciplinary Tumor Center, Mannheim University Medical Center, University of Heidelberg, Mannheim.
⁷ Department of Medical Oncology, Centre Oscar Lambret, Lille, France.
⁸ Department of Radiology, The Royal Marsden Hospital, London.
⁹ Division of Clinical Studies, The Institute of Cancer Research, London, UK.
¹⁰ Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: [email protected].

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.

Publication types

Practice Guideline
Review

MeSH terms

Fibromatosis, Aggressive / diagnosis*
Fibromatosis, Aggressive / genetics
Fibromatosis, Aggressive / therapy*
Humans
Randomized Controlled Trials as Topic