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mesenteric desmoid fibromatosis

Thursday 10 January 2013

Definition: Desmoid-type fibromatosis (desmoid) is a fibroblastic tumour that shows locally aggressive growth. Mesenteric desmoid is a rare lesion that shares morphological and biological features with fibromatoses occurring in the abdominal wall or in extraabdominal sites, but differs in terms of gross appearance and clinical presentation.

β-Catenin-negative desmoids either carried a CTNNB1 mutation or are associated with Gardner syndrome.

Some clinical and genetic features of mesenteric desmoids differ from those of non-mesenteric fibromatosis, and corroborates the usefulness of mutational analysis, especially in diagnosing β-catenin-negative mesenteric desmoids.

Reference

- β-Catenin (CTNNB1) mutations and clinicopathological features of mesenteric desmoid-type fibromatosis. Huss S, Nehles J, Binot E, Wardelmann E, Mittler J, Kleine MA, Künstlinger H, Hartmann W, Hohenberger P, Merkelbach-Bruse S, Buettner R, Schildhaus HU. Histopathology. 2013 Jan;62(2):294-304. doi : 10.1111/j.1365-2559.2012.04355.x PMID: 23020601