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

@article{Gronchi2014SporadicDF,
  title={Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group.},
  author={Alessandro Gronchi and Chiara Colombo and C{\'e}cile Le P{\'e}choux and Angelo P Dei Tos and Axel Le Cesne and Andrea Marrari and Nicolas Penel and Giovanni Grignani and Jean-Yves Blay and Paolo Giovanni Casali and Eberhard Stoeckle and Franco Gherlinzoni and P J H M Meeus and Cl{\'a}udia Mota Mussi and F. Gouin and Florence Duffaud and Marco Fiore and Sylvie Bonvalot},
  journal={Annals of oncology : official journal of the European Society for Medical Oncology},
  year={2014},
  volume={25 3},
  pages={578-83}
}
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… CONTINUE READING
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