The Desmoid Tumor Research Foundation

July 2017

Adult desmoid tumors: biology, management and ongoing trials.

Author Information

Frédéric Chibon,Nicolas Penel,Sébastien Salas

aDepartment of Medical Oncology, Centre Oscar Lambret, Lille bDepartment of Biopathology, Institut Bergonié, Comprehensive Cancer Centre, Institut National de la Santé et de la Recherche Medicale (INSERM) U916, Bordeaux cDivision of Adult Oncology, Department of Medicine, Aix-Marseille Université, INSERM U910 and Timone Hospital, Marseille, France.

Publication

Summary

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.

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