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Keywords
Endosonography, familial adenomatous polyposis (FAP),tubulopapillary adenoma.
Introduction
A 60-year-old female with familial adenomatous polyposis (FAP) presented with an ampullary mass which ultimately was shown to be a bile duct tubulopapillary adenoma. The findings and management are discussed.
Case/Body
A 60-year-old female was diagnosed with FAP forty years ago. She had a total colectomy with ileostomy at the time of diagnosis. She was doing well until twenty years ago when she required partial bowel resection for obstruction. Her past medical history included hypertension and a prior cholecystectomy. Family history was significant for a son and two grandchildren with FAP. She was taking Celecoxib 200mg four times daily for polyp chemoprevention. Physical exam was notable for lower extremity fibromas and an intact ileostomy. Laboratory studies were unremarkable. She had many surveillance endoscopies with polypectomies of duodenal polyps. Esophagogastroduodenoscopy (EGD) revealed multiple polyps in the fundus, duodenum and an ampullary lesion (Figure 1). A biopsy of the ampullary lesion revealed a tubular adenoma. CT scan noted a soft tissue swelling in the periampullary region of duodenum and an 8 x 8 cm adrenal mass. She was subsequently referred for endoscopic ultrasound (EUS) to evaluate the extent of the ampullary lesion. (Figures 2 and 3). EUS showed an ampullary lesion measuring 2 x 3 cm with no clear interface between the lesion and the pancreatic head parenchyma suggesting infiltration into the pancreas. These findings were suspicious for malignancy within this lesion. Patient proceeded to have pancreaticoduodenectomy (modified Whipple), and adrenal resection. Surgical pathology revealed an intraductal tubulopapillary adenoma, intestinal type of the common bile duct, that was compressing the pancreatic head. There were multifocal adenomatous changes involving duodenum and jejunum with multifocal glandular dysplasia, and an adrenal cortical adenoma.
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Discussion
FAP is an autosomal dominant disease which occurs in about 1:10000 people. It is due to a mutation in the APC gene. Colon cancer is inevitable in these patients. Various other cancers including ampullary carcinoma, thyroid, hepatoblastoma, gastric and CNS tumors have also been associated (1). Upper endoscopic surveillance is recommended. Surgery is proposed for those with malignancy (2). Management is controversial in those with dysplasia. Pancreaticoduodenectomy, surgical ampullectomy or endoscopic ampullectomy can be performed. The recurrence rate is higher (near 30%) with endoscopic resection compared to pancreaticoduodenectomy (3,4). The high recurrence with endoscopic resection could be due to the extension of the ampullary lesion into surrounding structures. EUS can help in delineating the extent of the lesion. In our patient, EUS suggested infiltration of the lesion into the pancreatic head making it suspicious for malignancy. As a result, we referred our patient for pancreaticoduodenectomy as opposed to endoscopic resection.
EUS may be useful in patients prior to endoscopic resection of ampullary lesions to evaluate the extent of the lesion. Our patient had a common bile duct adenoma which is a rare tumor. The natural history and malignant potential of these adenomas is unclear, therefore resection would be recommended. Fletcher et al., reported a case of a papillary adenoma which presented as lesion in the head of the pancreas (5). Similarly in our patient, since this lesion originated in the bile duct and was compressing on the pancreas, it masqueraded on EUS as an ampullary neoplasm infiltrating into the pancreatic parenchyma. Nevertheless, the EUS findings were quite helpful in determining that this lesion may not be amenable to endoscopic resection.
References
1.Giardiello FM, Brensinger JD, Petersen GM. AGA technical review on hereditary colorectal cancer and genetic testing. Gastroenterology. Jul 2001;121(1):198-213.
2. American Society for Gastrointestinal Endoscopy. The role of endoscopy in the surveillance of premalignant conditions of the upper gastrointestinal tract. Gastrointest Endosc. 1998 Dec;48(6):663-8.
3. Binmoeller KF, Boaventura S, Ramsperger K, Soehendra N. Endoscopic snare excision of benign adenomas of the papilla of Vater. Gastrointest Endosc. Mar-Apr 1993;39(2):127-31.
4.Catalano MF, Linder JD, Chak A, et al. Endoscopic management of adenoma of the major duodenal papilla. Gastrointest Endosc. Feb 2004;59(2):225-32.
5.Fletcher ND, Wise PE, Sharp KW. Common bile duct papillary adenoma causing obstructive jaundice: case report and review of the literature. Am Surg. May 2004;70(5):448-52.
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