Intra-Abdominal and Abdominal Wall Desmoid Fibromatosis.

ABSTRACT: Desmoid fibromatosis is a rare but locally aggressive tumor comprised of myofibroblasts. Desmoids do not have the ability to metastasize but can cause significant morbidity and mortality by local invasion. These tumors may occur throughout the body, but are commonly found on the abdominal wall and within the intestinal mesentery. Desmoids in these areas may cause unique clinical problems for physicians and patients. Mutations in either the β-catenin or the APC genes are usually the cause for the development of these tumors with the former comprising the sporadic development of tumors and the latter being associated with familial adenomatous polyposis syndrome. Surgical resection with histologically negative margins has been the cornerstone of therapy for this disease, but this parad

SUBMITTER: Howard JH 

PROVIDER: S-EPMC5315078 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature