Adult desmoid tumors: biology, management ...
Type de document :
Article dans une revue scientifique: Article de synthèse/Review paper
PMID :
URL permanente :
Titre :
Adult desmoid tumors: biology, management and ongoing trials
Auteur(s) :
Penel, Nicolas [Auteur]
Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
Chibon, Frédéric [Auteur]
Salas, Sébastien [Auteur]
Evaluation des technologies de santé et des pratiques médicales - ULR 2694 [METRICS]
METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694
Chibon, Frédéric [Auteur]
Salas, Sébastien [Auteur]
Titre de la revue :
Current opinion in oncology
Nom court de la revue :
Curr. Opin. Oncol.
Numéro :
29
Pagination :
268-274
Date de publication :
2017-07-01
ISSN :
1040-8746
Mot(s)-clé(s) en anglais :
wait and see
desmoid tumor
CTNNB1
APC
desmoid tumor
CTNNB1
APC
Discipline(s) HAL :
Sciences du Vivant [q-bio]
Résumé en anglais : [en]
OBJECTIVE: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors.
RESULTS: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/β-catenin ...
Lire la suite >OBJECTIVE: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors. RESULTS: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/β-catenin pathway, sporadic desmoid tumors are associated with somatic mutations of CTNNB1, and germline mutations of APC and somatic mutations of CTNNB1 are probably mutually exclusive. One-third of desmoid tumors are misdiagnosed; a second pathological opinion is therefore of major importance for desmoid tumor. Surgery is no longer regarded as the cornerstone of desmoid tumors; several retrospective studies have demonstrated the safety of a 'wait and see' policy in sporadic abdominal wall desmoid tumor. Desmoid tumors is no longer regarded as an absolute contraindication for pregnancy. At least two new investigational drugs targeting the Wnt/APC/β-catenin pathway are currently being developed. CONCLUSIONS: The management of desmoid tumors requires multidisciplinary expertise by an experienced team. We must fully understand the physiopathology of the disease (factors influencing the natural history of the disease) and learn how to avoid desmoid tumors occurrence in patients with APC germline mutations, identify reliable prognostic/predictive factors and better assess the efficacy of systemic treatment.Lire moins >
Lire la suite >OBJECTIVE: To summarize the current knowledge about the biology and clinical management of adult desmoid tumors. RESULTS: In the past decade, we have learned that desmoid tumors are driven by alterations of the Wnt/APC/β-catenin pathway, sporadic desmoid tumors are associated with somatic mutations of CTNNB1, and germline mutations of APC and somatic mutations of CTNNB1 are probably mutually exclusive. One-third of desmoid tumors are misdiagnosed; a second pathological opinion is therefore of major importance for desmoid tumor. Surgery is no longer regarded as the cornerstone of desmoid tumors; several retrospective studies have demonstrated the safety of a 'wait and see' policy in sporadic abdominal wall desmoid tumor. Desmoid tumors is no longer regarded as an absolute contraindication for pregnancy. At least two new investigational drugs targeting the Wnt/APC/β-catenin pathway are currently being developed. CONCLUSIONS: The management of desmoid tumors requires multidisciplinary expertise by an experienced team. We must fully understand the physiopathology of the disease (factors influencing the natural history of the disease) and learn how to avoid desmoid tumors occurrence in patients with APC germline mutations, identify reliable prognostic/predictive factors and better assess the efficacy of systemic treatment.Lire moins >
Langue :
Anglais
Audience :
Internationale
Vulgarisation :
Non
Établissement(s) :
CHU Lille
Université de Lille
Université de Lille
Date de dépôt :
2019-12-09T18:16:45Z