纤维瘤病是一种罕见的局部侵袭性单克隆，成纤维细胞增殖的特征是可变的，通常是不可预测的临床过程。目前,目前尚无针对该疾病的既定或循证治疗方法.因此,2015年,欧洲Desmoid工作组发表了一份立场文件,对这种有趣疾病的治疗提出了建议.这里,在来自欧洲癌症研究和治疗组织/软组织和骨肉瘤组的肉瘤专家与来自肉瘤患者EuroNet的患者和患者倡导者的圆桌会议之后,我们基于专业人员和患者的专业知识,提出了这一共识方法的更新.在本文中,我们关注突变分析在纤维瘤样病变型患者中的预后价值方面的新发现,以及新的全身治疗方案.
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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