BACKGROUND: Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize. The choice of treatment remains surgical resection with or without radiotherapy, and is characterized by high recurrence rates. Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates. METHODS: Relevant studies were identified through a search of the electronic databases PubMed/ Medline. The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'. Studies were selected for review on the basis of abstract reading. A hand search was performed by checking reference lists in selected articles. RESULTS: The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed. CONCLUSION: Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors. More research is needed to develop new treatment strategies.
BACKGROUND:Aggressive fibromatosis (syn. desmoid tumor) is a sporadically occurring neoplastic proliferation of fibroblasts originating from musculoaponeurotic planes, forming invasively growing masses without the capability to metastasize. The choice of treatment remains surgical resection with or without radiotherapy, and is characterized by high recurrence rates. Better understanding of the aetiology of aggressive fibromatosis is needed to be able to develop new treatment strategies to cope with the high recurrence rates. METHODS: Relevant studies were identified through a search of the electronic databases PubMed/ Medline. The following search terms were used: 'aggressive fibromatosis', 'desmoid tumor', 'adenomatous polyposis coli', 'APC', 'beta-catenin', 'Wnt', 'Wingless' and 'Wnt/Wingless'. Studies were selected for review on the basis of abstract reading. A hand search was performed by checking reference lists in selected articles. RESULTS: The neoplastic nature of aggressive fibromatosis and the role of the adenomatous polyposis coli (APC) and beta-catenin signaling cascade in driving the onset and progression of this disease are discussed. CONCLUSION: Mutations in either the APC or beta-catenin genes are likely to be a major driving force in the formation of these desmoid tumors. More research is needed to develop new treatment strategies.
Authors: Mohamed Allaoui; Mohamed Tarchouli; Adil Boudhas; Reda El Ochi; Ahmed Bounaim; Abderrahmane Al Bouzidi; Mohamed Oukabli Journal: J Gastrointest Cancer Date: 2018-03
Authors: F Bremmer; C L Behnes; H U Schildhaus; N T Gaisa; H Reis; H Jarry; H J Radzun; P Stroebel; S Schweyer Journal: Virchows Arch Date: 2017-02-16 Impact factor: 4.064
Authors: Maria Teresa Vietri; Vietri Maria Teresa; Francesco Selvaggi; Selvaggi Francesco; Maria Laura De Paola; De Paola Maria Laura; Guido Sciaudone; Sciaudone Guido; Ilaria Guadagni; Guadagni Ilaria; Mariarita Parisi; Parisi Mariarita; Gianluca Pellino; Pellino Gianluca; Anna Maria Molinari; Molinari Anna Maria; Michele Cioffi; Cioffi Michele Journal: J Mol Genet Med Date: 2010-05-13