Ampulla of Vater

The ampulla of Vater, also hepatopancreatic ampulla, is found in the duodenum. It has a unique histology and is a relatively common site of disease, when duodenal pathology is considered.

Benign

Normal histology

Periampullary:

• Intestinal epithelium with goblet cells.

Papilla of Vater (the projection into the duodenal lumen):

• Goblet cells in foveolar-like epithelium.

Note about heterotopias:

• +/-Pancreatic heterotopia - common.
• +/-Gastric heterotopia - not common.

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DUODENAL BULB, BIOPSY:
- GASTRIC HETEROTOPIA.
- NEGATIVE FOR MALIGNANCY.

Acute inflammation

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Ampulla of Vater, Biopsy:
- Small bowel mucosa with foveolar-like glands (in keeping with 
  ampulla), reactive epithelial changes and mild acute inflammation.
- NEGATIVE for dysplasia.

Ampullary tumours

• AKA tumours of the ampulla of Vater.
• AKA tumours of the hepatopancreatic ampulla.

Ampullary Obstruction

Villous appearing duodenal mass of ampulla, secondary to ampullary duct obstruction. 43 yo White man. A. Plicae show expansion of stroma by edema with inflammation including neutrophils (neutrophils not shown), by foci of lamina propria hemorrhage (red arrows), and by dilated lymphatics (black arrows). B. A fragment shows dilated ducts on left and center, with connected “proliferating” ducts at right. C. PAS AB stain shows these ducts, by virtue of the thin red line, are ampullary, not Brunner’s glands. D. Nuclei in the proliferated area show striking anisokaryosis and anisonucleosis, as well as hyperchromasia. Note the mitosis (arrow). This notwithstainging, there is an appearance of connection between the ducts. No net appears. Neither are there half glands or aberrant isolated cells. E. On PAS AB stain lay a focus of fibrinopurulent exudate near the proliferating area.

Ampullary adenoma

General

• Uncommon.
• May be associated with familial adenomatous polyposis (FAP).

Microscopic

Features:

• +/-Paneth cells - may be prominent.
• Similar to adenoma of colon - with:
  • Less pseudostratification.
  • Finer chromatin pattern.

DDx:

• Ampullary carcinoma.
• Duodenal carcinoma secondarily involving the ampulla.
• Pancreatic adenocarcinoma secondarily involving the ampulla.

Images

www:

• Ampullary adenoma (ajcpjournals.org).
• Ampullary adenoma - pitfalls (ajcpjournals.org).

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See tubular adenoma.

Ampullary carcinoma

• AKA ampullary adenocarcinoma.

General

• Uncommon.
• Textbook association: familial adenomatous polyposis.
• Prognosis guarded but significantly better than pancreatic ductal adenocarcinoma - 5 year survival ~40% for ampullary carcinomas vs. 10% for pancreatic adenocarcinoma.

Gross

• Ampullary carcinomas are classified by site.
• Modest differences exist in survival between the sites.

Classification

Adsay et al. proposed a four subtype classification system:

Subtype	Prevalence	Origin/definition	Notes
Intra-ampullary papillary-tubular carcinoma	~25% of cases	arises from intra-ampullary epithelium and/or distal end of CBD or pancreatic duct	generalized category intra-ampullary papillary-tubular neoplasm -- the ampullary counterpart of the IPMN of the pancreas
Ampullary-ductal carcinoma	~15% of cases	arises from intra-ampullary ducts
Peri-ampullary duodenal carcinoma	~5% of cases	primarily in the duodenum; ampullary orfice must be clearly within lesion
Ampullary carcinoma not otherwise specified	~55% of cases	arise from papillary projection into duodenum - from foveolar-like epithelium with goblet cells

Microscopic

Dependent on histologic subtype:

1. Intestinal ampullary carcinoma.
2. Pancreaticobiliary ampullary carcinoma.
3. Other.

Invasive carcinoma of duodenal ampulla, intestinal type. A. Ulceration tops Brunner’s glands [green arrow]; at the edge lie glands [blue arrow] with changes suggesting adenoma (100X). B. Ki67 stain establishes adenoma by surface extension of brown nuclei (100X). C. The lesion was a mass, prompting rebiopsy. The cauterized fragment shows disorderly spread of glands, with dilated glands at base [arrows] not readily explained by obstruction (40X) D.Two cancerous prongs, one on left [black arrow], one on right [blue arrow] each show spread about muscle fibers (200X). E. The Whipple resection showed the same dilated spreading glands [arrows] at base, redolent of the spread of some well-differentiated colonic adenocarcinomas through muscle (40X). F. Cribriformed cancerous nests [arrows] cannot be Brunner’s glands, because the nuclei are too variable and lack polarity and because they abut crypts extending to the surface; cautery would have made this an impossible distinction (200X).

60 year old woman with poorly differentiated ampullary adenocarcinoma and obstructive cholestasis in liver. A. An isolated tumor fragment (black arrow) and two fragments with tumor undermining small intesatinal mucosa (green arrows) are seen. B. Large, ovoid cancer nuclei with abundant grey cytoplasm form sheets with only rare acini (arrow). C. Tumor cells are CK7 positive. D. Tumor cells are CK20 negative. E. Portal triads (black arrows) are expanded, lacking intense inflammation. A septal duct (green arrow) lacks definite onion skinning. F. Proliferated peripheral bile ductules (black arrows) sometimes have neutrophils (white arrow), which should not be interpreted as ascending cholangitis. Next to the artery (red arrow) lies the bile duct (yellow arrow), which has disturbed nuclei, but no neutrophils. Note occasional eosinophils (cyan arrows). G. Hepatocytes with feathery degeneration. Arrows point to dilated cholangioles, one filled with a bile plug.

Notes:

• May lack desmoplastic stroma.

DDx:

• Invasive ductal carcinoma of the pancreas - has a much worse prognosis.
• Ectopic pancreas.
• Duodenal adenocarcinoma with secondary involvement of the ampulla.

Intestinal ampullary carcinoma

Features:

• Pseudostratified columnar epithelium with hyperchromatic, ellipsoid nuclei.
  • Glands usually tightly packed, i.e. high gland-to-stroma ratio; often >2:1.

Note:

• Similar to colorectal adenocarcinoma.

Pancreatobiliary ampullary carcinoma

Features:

• Tubular arrangements consisting of cuboidal cells in one or two layers.
  • Tubules usually spaced; ~1:3 gland-to-stroma ratio.

Images

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  PA - low mag. (WC)

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  PA - intermed. mag. (WC)

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  PA - high mag. (WC)

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  PA - high mag. (WC)

Other

Features - any of the following characteristics:

• Non-tubular morphology/poorly-differentiated.
• Micropapillary architecture.
• Medullary.
• Signet ring cells.
• Mucin:
  1. Colloid.
  2. Mixed-mucinous.
  3. Mucinous-signet-ring.

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  Ampulla Ampullary Adeno Carcinoma -(SKB)

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  Ampulla Ampullary Adeno Carcinoma - (SKB)

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  Ampulla Ampullary Adeno Carcinoma - (SKB)

IHC

Features:

• CK7 +ve.
• CK20 +ve.
• MUC2 +ve.

Others:

• SMAD4 +ve/-ve.
  • Lost in pancreatic neoplasia ~90% of cases vs. ~35% of ampullary tumours.
• p53 +ve ~55% of cases.
• E-cadherin +ve ~40% of cases.
• Beta-catenin +ve ~65% of cases.

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• Separate CAP protocol.

See also

• Duodenum.
• Small bowel.
• Gastrointestinal pathology.