Ampullary Adenocarcinoma and Variants

 Intraampullary: Arise in ampulla (lined by pancreatobiliary epithelium)

image Periampullary: Arise in duodenal surface of papilla (lined by intestinal epithelium)

Clinical Issues

• 5-year overall survival rate: 40%
image Determined by histologic type, grade, stage, coexisting adenoma


• 2 main histologic types
image Intestinal-type adenocarcinoma

– Most common (50-80%)

– Similar to adenocarcinoma of colon and small bowel

image Pancreatobiliary-type adenocarcinoma

– 2nd most common (15-20%)

– Similar to pancreatic ductal or bile duct adenocarcinoma

Ancillary Tests

• Intestinal type
image CK20, CDX-2, or MUC2 (+), MUC1(-)

image CK20, CDX-2, and MUC2 (+), irrespective of MUC1

• Pancreatobiliary type
image MUC1(+), CDX-2 and MUC2 (-)

Top Differential Diagnoses

• Adenocarcinomas of pancreas, distal common bile duct, and duodenum

Diagnostic Checklist

• Important to distinguish intestinal type from pancreatobiliary type because of more favorable prognosis

Ampullary Mass
From the luminal aspect of this resection specimen, the ampulla is replaced by an exophytic, ulcerated mass image involving the papilla and periampullary duodenal mucosa. Histologic examination confirms the diagnosis of ampullary adenocarcinoma.

Ampullary Mass
Ampullary adenocarcinoma presents as an exophytic, white mass obstructing the orifice image of the common bile duct image, which is dilated proximally. The pancreatic duct image is not obstructed.

Intestinal Type
This intestinal-type ampullary adenocarcinoma shows a cribriform pattern with luminal “dirty necrosis” image. Tumor cells are columnar with stratified, elongated or oval nuclei, resembling adenocarcinoma of colon and small bowel.

CDX2 Immunostain
Tumor cells in this ampullary adenocarcinoma are positive for CDX-2. They are also positive for CK20 and MUC2 and only focally positive for MUC1 (in < 10% of tumor cells). This immunohistochemical profile is consistent with intestinal type.



• Periampullary adenocarcinoma


• Malignant epithelial neoplasm originating in ampulla of Vater
image Centered on, circumferentially surrounding, or completely replacing ampulla



• Intraampullary: Arise in ampulla (lined by pancreatobiliary epithelium)

• Periampullary: Arise in duodenal surface of papilla (lined by intestinal epithelium)

Predisposing Syndromes

• Familial adenomatous polyposis, Lynch syndrome, type 1 neurofibromatosis



• Incidence
image 0.7 and 0.4 cases per 100,000 males and females, respectively

image 0.5% of all gastrointestinal malignancies

• Age
image Mean: 62 years (range: 29-85 years)
– Younger in patients with predisposing syndromes


• Jaundice, abdominal pain, pancreatitis, weight loss


• Resection (Whipple procedure)

• Chemoradiation therapy

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Ampullary Adenocarcinoma and Variants

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