期刊
ANNALS OF ONCOLOGY
卷 28, 期 10, 页码 2399-2408出版社
OXFORD UNIV PRESS
DOI: 10.1093/annonc/mdx323
关键词
desmoid; aggressive fibromatosis; EORTC/STBSG; patient advocacy groups; SPAEN; treatment algorithm
类别
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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