A Sporadic Giant Mesenteric Desmoid-Type Fibromatosis in a Teenage Female: A Report of a Rare Case

Summary

Mesenteric desmoid-type fibromatosis (DTF) is a rare benign yet aggressive neoplasm that has an unpredictable biological behavior ranging from spontaneous regression to extensive local infiltration and has a high tendency for recurrence. The presenting symptoms are usually nonspecific and mostly related to the large size of the tumor compressing adjacent organs. Imaging studies can be suggestive of the diagnosis, but confirmation is based on histopathological and immunohistochemical examination. The lack of knowledge on the etiology and pathogenetic behavior of this tumor leads to therapeutic and prognostic challenges. Future genetic studies may help in advancing our understanding of this neoplasm and in formulating the proper management and follow-up plan. Here we present a case of a 14-year-old female who presented to the emergency room complaining of diffuse abdominal pain and distention. A computed tomography (CT) scan showed a large mass occupying most of the abdominal cavity and compressing adjacent organs. Exploratory laparotomy with resection and anastomosis was performed, and the histopathological and immunohistochemical examination of the resected mass was consistent with mesenteric DTF.