Fibromatosis of the Remnant Pancreas After Pylorus-Preserving Pancreaticoduodenectomy

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 wi...

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Bibliographic Details
Published in:Journal of gastrointestinal surgery 2006-05, Vol.10 (5), p.679-688
Main Authors: Weiss, Eric S., Burkart, Ashlie L., Yeo, Charles J.
Format: Article
Language:English
Subjects:
Colorectal cancer
desmoid
Fibroma - pathology
fibromatosis
Fibromatosis, Aggressive - pathology
Humans
Male
Middle Aged
Pancreas
Pancreas - pathology
Pancreas - surgery
pancreaticoduodenectomy
Pancreaticoduodenectomy - methods
Pylorus - surgery
Tomography, X-Ray Computed - methods
Tumors
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Summary: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.
ISSN:1091-255X
1873-4626
DOI:10.1016/j.gassur.2005.09.029