Mesenteric fibromatosis with intestinal involvement mimicking a gastrointestinal stromal tumour

Abstract

IntroductionMesenteric fibromatosis or intra-abdominal desmoid tumour is a rare proliferative disease affecting the mesentery. It is a locally aggressive tumour that lacks metastatic potential, but the local recurrence is common. Mesenteric fibromatosis with the intestinal involvement can be easily confused with other primary gastrointestinal tumours, especially with that of the mesenchymal origin.Case reportWe report a case of a 44-year-old female who presented with an abdominal mass that radiologically and pathologically mimicked a gastrointestinal stromal tumour.ConclusionsThe diagnosis of mesenteric fibromatosis should always be considered in the case of mesenchymal tumours apparently originating from the bowel wall that diffusely infiltrate the mesentery.

Desmoid-type Fibromatosis of the Mesentery: A Case Report

“…Clinically, DTF are presented by a slow abdominal enlargement and various symptoms such as compression, intestinal obstruction or circulatory disorders, associated with ischemia and hemorrhages due to intestinal perforation [2][3][4]. In this case, the initial abdominal enlargement was asymptomatic but later on subileal manifestations, perforation and peritonitis were found.…”

“…Most DTF cases are sporadic, and in 80-90% of them a somatic mutation of the Adenomatous Polyposis Coli gene (APC) is determined, which activates a mutation in Catenin Beta 1 gene ( NN 1), which results in accumulation of -catenin and turns out to be the triggering mechanism of the broblastic proliferation [2,3,5].…”

“…However, their biological behavior, such as in ltrative growth and local recurrence, has provided reasons for some authors to refer to them as intermediate malign neoplasms [2,3,5].…”

“…The latter are subcategorized as mesenteric bromatoses (MF), mesenteric bromatoses associated with Gardner's syndrome, and pelvic bromatoses [3][4][5].…”

Mesenteric fibromatosis: a rare cause of peritonitis

“…It is a histologically benign disease and lacks the capacity to metastasise. [1][2][3] Nonetheless, MF is locally aggressive with a high recurrence rate after surgical resection. Symptomatic MF mostly presents with abdominal pain or a palpable mass on physical examination.…”