Sporadic Hereditary Pancreatic Desmoid Tumor

Abstract

Deeply seated aggressive fibromatosis also termed as desmoid tumors are rare tumors that invade surrounding structures. Although they never metastasize mortality rate may be as high as 10% due to their aggressive local behavior. Intraabdominal desmoid tumors are usually associated with familial poliposis coli and have a high recurrence rate regardless of the therapy instituted. Sporadic cases are very rare and generous surgical excision may be of benefit. We hereby report 2 siblings with sporadic pancreatic desmoid tumors who also harbor additional fibrotic masses in the pelvis. Although in previously reported cases there is usually a triggering event such as trauma, in the present cases there was no inciting event. Furthermore, the cases are without an associated FAP history, which provides the first clinical clue of a possible genetic determinant in this rare disorder.