Ampullary Adenocarcinoma and Variants
Mari Mino-Kenudson, MD
Key Facts
Terminology
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Ampullary adenocarcinoma comprises both adenocarcinomas arising in ampullary region and periampullary duodenal adenocarcinomas
Clinical Issues
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5-year survival rate after surgical resection is approximately 50%
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Significantly better than that of pancreatic adenocarcinoma
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Macroscopic Features
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Tumors may be intraampullary, periampullary duodenal, mixed exophytic, or mixed ulcerated
Microscopic Pathology
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Intestinal type
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Pancreatobiliary type
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Papillary carcinoma (noninvasive)
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Invasive papillary carcinoma
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Mucinous (colloid) carcinoma
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Adenosquamous carcinoma
Ancillary Tests
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Intestinal type usually positive for CK20 and CDX2; often negative for CK7
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Pancreatobiliary type usually positive for CK7; often negative for CK20 and CDX2
Diagnostic Checklist
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Intestinal type of histologic differentiation is associated with favorable outcome in comparison to pancreatobiliary type
TERMINOLOGY
Synonyms
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Periampullary adenocarcinoma
Definitions
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Adenocarcinoma arising in ampullary region and periampullary duodenal adenocarcinoma are collectively termed “ampullary adenocarcinoma”
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Approximately 90% of all carcinomas of region
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CLINICAL ISSUES
Epidemiology
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Incidence
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Relatively uncommon
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Approximately 0.2% of GI tract malignancies
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Ampulla is most common site of small bowel adenocarcinoma
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Age
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Most common in 7th-8th decade of life
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Patients with familial adenomatous polyposis develop ampullary carcinoma at younger age than patients with sporadic cases
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Gender
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Slightly more common in men (M:F = 1.48:1)
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Presentation
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Jaundice
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Weight loss
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Abdominal pain
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Distended, palpable gallbladder (Courvoisier sign)
Treatment
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Resection (Whipple procedure)
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Resectability is approximately 60%
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Role of adjuvant chemoradiation therapy (5-FU based) is controversial
Prognosis
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5-year survival rate after surgical resection is approximately 50%
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Significantly better than that of pancreatic adenocarcinoma
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Comparable to that of duodenal adenocarcinoma
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