title: Sporadic desmoid-type fibromatosis: a stepwise approach to a non-metastasising neoplasm--a position paper from the Italian and the French Sarcoma Group.
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.
authors: Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy. Electronic address: alessandro.gronchi@istitutotumori.mi.it.
authors: A
authors: A
authors: Gronchi
authors: Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: C
authors: C
authors: Colombo
authors: Department of Radiation Oncology, Institute Gustave Roussy, Villejuif, France.
authors: C
authors: C
authors: Le Péchoux
authors: Department of Pathology, Treviso General Hospital, Treviso, Italy.
authors: A P
authors: AP
authors: Dei Tos
authors: Department of Cancer Medicine, Institute Gustave Roussy, Villejuif, France.
authors: A
authors: A
authors: Le Cesne
authors: Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: A
authors: A
authors: Marrari
authors: Department of General Oncology, Centre Oscar Lambret, Lille, France.
authors: N
authors: N
authors: Penel
authors: Department of Cancer Medicine, Institute for Cancer Research and Treatment at Candiolo, Torino, Italy.
authors: G
authors: G
authors: Grignani
authors: Department of Cancer Medicine, Centre Leon Berard, Lyon.
authors: J Y
authors: JY
authors: Blay
authors: Department of Cancer Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: P G
authors: PG
authors: Casali
authors: Department of Surgery, Institut Bergonié, Regional Cancer Centre, Bordeaux, France.
authors: E
authors: E
authors: Stoeckle
authors: Department of Orthopedics, Gorizia General Hospital, Gorizia, Italy.
authors: F
authors: F
authors: Gherlinzoni
authors: Department of Surgery, Centre Leon Berard, Lyon, France.
authors: P
authors: P
authors: Meeus
authors: Department of Surgery, Humanitas Cancer Center, Rozzano, Italy.
authors: C
authors: C
authors: Mussi
authors: Department of Orthopedics, Centre Hospitalier Universitaire, Nantes.
authors: F
authors: F
authors: Gouin
authors: Department of Medical Oncology, Marseille and Aix-Marseille University, Marseille.
authors: F
authors: F
authors: Duffaud
authors: Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
authors: M
authors: M
authors: Fiore
authors: Department of Surgery, Institute Gustave Roussy, Villejuif, France.
authors: S
authors: S
authors: Bonvalot
mesh_terms: D018222:Desmoid Tumors / Q000235:genetics / Q000532:radiotherapy* / Q000601:surgery*; D005602:France; D006801:Humans; D007558:Italy; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy; D057832:Watchful Waiting; D060449:Wnt Signaling Pathway / Q000235:genetics; D051176:beta Catenin / Q000235:genetics*
chemical_list: C495270:CTNNB1 protein, human; D051176:beta Catenin
keywords: aggressive fibromatosis; desmoid tumor; medical therapy; outcome; radiation therapy; surgery; wait and see approach