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)

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.

Clinical benefit of methotrexate plus vinorelbine chemotherapy for desmoid fibromatosis (DF) and correlation of treatment response with MRI

“…When there is persistent progression of DF, many expert centers currently advocate for the use of medical therapy for initial intervention . Although there are several systemic therapeutic DF options, in the absence of comparative studies, most institutions have selected a regimen based on historical data and local experience.…”

Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: a literature review and focus group study

“…Although such end-points can be appropriate in malignant diseases, the unpredictable growth behaviour including spontaneous regression and the low mortality rate of sporadic DTF renders such outcomes less appropriate for this borderline disease [16]. Consequently, the question rises whether health-related quality of life (HRQL) assessment could be a more appropriate outcome measure in DTF [10, 14, 19, 20]. The definition of HRQL is “a patients’ evaluation of the impact of a health condition and its treatment on all relevant aspects of life”.…”

Toward a new strategy in desmoid of the breast?

“…This is notable as surgery was demonstrated to be very disfiguring in the previous period, and 40% of patients ultimately underwent mastectomy or chest wall resection for a benign tumor. Medical treatment was able to stop a number of progressions and is recently proposed in a stepwise approach, 15,16 but it is difficult to clearly attribute this stabilization to the medical treatment or to the natural history of the tumor given the lack of randomization.…”