Fibromatosis of the remnant pancreas after pylorus-preserving pancreaticoduodenectomy

Abstract

Intra-abdominal fibromatosis or desmoid tumors are rare forms of connective tissue cellular dysplasia characterized by proliferation of fibroblasts and abundant collagen. Most often these tumors associated with familial adenomatous polyposis or Gardner’s syndrome. Those tumors not associated with polyposis are termed sporadic desmoids and tend to be locally aggressive in nature. Sporadic intra-abdominal desmoids involving the pancreas are quite rare, as only six previously reported cases exist. In this report we present a seventh case of a sporadic intraabdominal desmoid involving the pancreas. The patient, a 63-year-old white man, developed the desmoid tumor following a pylorus-preserving pancreaticoduodenectomy for an insulinoma. The patient was managed via further pancreatectomy, consisting of a distal pancreatectomy with en bloc splenectomy, sparing a 6-cm portion of pancreatic neck and proximal body. Finally, we present a complete review of the six previous cases of sporadic pancreatic fibromatosis.