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).

No Thumbnail Available

Embargo End Date

Authors

Kasper, B
Baumgarten, C
Garcia, J
Bonvalot, S
Haas, R
Haller, F
Hohenberger, P
Penel, N
Messiou, C
van der Graaf, WT
Gronchi, A
Desmoid Working Group,

Document Type

Journal Article

Date

2017-10-01

Date Accepted

2017-06-13

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.

Citation

Annals of oncology : official journal of the European Society for Medical Oncology, 2017, 28 (10), pp. 2399 - 2408

Source Title

Publisher

OXFORD UNIV PRESS

ISSN

0923-7534

eISSN

1569-8041

Collections

Research Team

Clinical and Translational Sarcoma
Sarcoma Clinical Trials

Notes