NDLI: Abdominal desmoid in familial adenomatous polyposis presenting as a pancreatic cystic lesion

Content Provider	Springer Nature Link
Author	Pho, Lana N. Coffin, Cheryl M. Burt, Randall W.
Copyright Year	2005
Abstract	A 17-year-old male with familial adenomatous polyposis (FAP) presented with chest pain and significant weight loss. An abdominal CT scan detected a cystic pancreatic lesion of unknown etiology. The patient therefore underwent surgical resection of the distal pancreas, which included the lesion, because of the known association of pancreatic cancer with FAP. Histopathological examination of the resected specimen showed a benign pancreatic cyst and fibrous plaque with desmoid fibromatosis adherent to the surface of the pancreas, serosa of the stomach, and colon. The fibrous plaque was histologically identical to the fibrous mesenteric plaque known to occur in FAP and associated mesenteric fibromatosis. We present pathologic evidence that the pancreatic cyst formation was induced by FAP-associated desmoid invasion. Desmoid growth should be considered in the differential diagnosis of a pancreatic cystic mass lesion in patients with FAP or its Gardner syndrome variant. This case report provides the first pathologic evidence for benign epithelial cyst formation in the pancreas caused by fibromatosis invasion of that organ as a part of FAP.
Starting Page	135
Ending Page	138
Page Count	4
File Format	PDF
ISSN	13899600
Journal	Familial Cancer
Volume Number	4
Issue Number	2
e-ISSN	15737292
Language	English
Publisher	Kluwer Academic Publishers
Publisher Date	2005-01-01
Publisher Place	Dordrecht
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	desmoid familial adenomatous polyposis FAP Gardner syndrome pancreatic cyst Human Genetics Oncology Epidemiology
Content Type	Text
Resource Type	Article
Subject	Genetics Cancer Research Genetics (clinical) Oncology

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