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The Desmoid Tumor Research Foundation

October 2017

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)

Author Information

Kasper B(1), Baumgarten C(2), Garcia J(2), Bonvalot S(3), Haas R(4)(5), Haller F(6), Hohenberger P(1), Penel N(7), Messiou C(8), van der Graaf WT(9), Gronchi A(10); Desmoid Working Group.

(1)Sarcoma Unit, Interdisciplinary Tumor Center, Mannheim University MedicalCenter, University of Heidelberg, Mannheim.(2)SPAEN Sarcoma PAtients EuroNet e.V, Wšlfersheim, 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.(5)Department of Radiotherapy, Leiden University Medical Center, Leiden, The Netherlands.(6)Institute of Pathology, Friedrich Alexander University Erlangen, Erlangen, Germany.(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.

Publication

Ann Oncol

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.

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