Ampullary Adenocarcinoma and Variants

Mari Mino-Kenudson, MD

Key Facts

Terminology

Ampullary adenocarcinoma comprises both adenocarcinomas arising in ampullary region and periampullary duodenal adenocarcinomas

Clinical Issues

5-year survival rate after surgical resection is approximately 50%
- Significantly better than that of pancreatic adenocarcinoma

Macroscopic Features

Tumors may be intraampullary, periampullary duodenal, mixed exophytic, or mixed ulcerated

Microscopic Pathology

Intestinal type
Pancreatobiliary type
Papillary carcinoma (noninvasive)
Invasive papillary carcinoma
Mucinous (colloid) carcinoma
Adenosquamous carcinoma

Ancillary Tests

Intestinal type usually positive for CK20 and CDX2; often negative for CK7
Pancreatobiliary type usually positive for CK7; often negative for CK20 and CDX2

Diagnostic Checklist

Intestinal type of histologic differentiation is associated with favorable outcome in comparison to pancreatobiliary type

From the luminal aspect of this resection specimen, the ampulla is replaced by a protruding tumor grossly involving the duodenal mucosa of the papilla and periampullary duodenum.

This ampullary adenocarcinoma consists of an exophytic white tumor involving the orifice

of the common bile duct

, but not invading the pancreatic duct

TERMINOLOGY

Synonyms

Periampullary adenocarcinoma

Definitions

Adenocarcinoma arising in ampullary region and periampullary duodenal adenocarcinoma are collectively termed “ampullary adenocarcinoma”
- Approximately 90% of all carcinomas of region

CLINICAL ISSUES

Epidemiology

Incidence
- Relatively uncommon
  - Approximately 0.2% of GI tract malignancies
  - Ampulla is most common site of small bowel adenocarcinoma
Age
- Most common in 7th-8th decade of life
- Patients with familial adenomatous polyposis develop ampullary carcinoma at younger age than patients with sporadic cases
Gender
- Slightly more common in men (M:F = 1.48:1)

Presentation

Jaundice
Weight loss
Abdominal pain
Distended, palpable gallbladder (Courvoisier sign)

Treatment

Resection (Whipple procedure)
- Resectability is approximately 60%
Role of adjuvant chemoradiation therapy (5-FU based) is controversial

Prognosis

5-year survival rate after surgical resection is approximately 50%
- Significantly better than that of pancreatic adenocarcinoma
- Comparable to that of duodenal adenocarcinoma

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