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. 2014 Mar;25(3):578-583.
doi: 10.1093/annonc/mdt485. Epub 2013 Dec 9.

Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group

A Gronchi  1 , C Colombo  2 , C Le Péchoux  3 , A P Dei Tos  4 , A Le Cesne  5 , A Marrari  6 , N Penel  7 , G Grignani  8 , J Y Blay  9 , P G Casali  6 , E Stoeckle  10 , F Gherlinzoni  11 , P Meeus  12 , C Mussi  13 , F Gouin  14 , F Duffaud  15 , M Fiore  2 , S Bonvalot  16 ; ISG and FSG
Affiliations

Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group

A Gronchi et al. Ann Oncol. 2014 Mar.

Abstract

Desmoid-type fibromatosis (DF) is a rare locally aggressive monoclonal proliferation of myofibroblasts lacking metastatic capacity. It may be observed in nearly every part of the body. Considering the variable clinical presentations, anatomic locations, and biologic behaviors, an individualized treatment approach is required. The pathogenesis of DF is not completely understood even if a high prevalence (∼85%) of CTNNB1 mutations discovered in sporadic DF underlies the importance of the Wnt/β-catenin pathway. No established and evidence-based approach for the treatment of this neoplasm is available as of today. Considering the unpredictable behavior and the heterogeneity of this disease, we propose a treatment algorithm approved by the French and the Italian Sarcoma Group, based on a front-line wait and see approach and subsequent therapy in the case of progression. A careful counseling at a referral center is mandatory and should be offered to all patients affected by sporadic DF from the time of their diagnosis.

Keywords: aggressive fibromatosis; desmoid tumor; medical therapy; outcome; radiation therapy; surgery; wait and see approach.

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Figures

Figure 1.
Figure 1.
Different patterns of tumor growth according to the time point of first observation in the clinic.
Figure 2.
Figure 2.
Consensus algorithm of treatment of sporadic desmoid-type fibromatosis.
See this image and copyright information in PMC

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