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  • • Partial or complete obstruction of the common bile duct and pancreatic duct at the ampulla of Vater

    • Exophytic mass at the ampulla visible on endoscopy

    • Jaundice, abdominal pain, and weight loss may be presenting symptoms

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Epidemiology

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  • • Adenoma and adenocarcinoma of the ampulla of Vater account for about 10% of neoplasms that obstruct the distal bile duct

    • Of primary tumors of the ampulla of Vater, 33% are adenomas and 67% are adenocarcinomas

    • It is suspected that malignant change in an adenoma gives rise to most carcinomas, and adenomas may contain focus of adenocarcinoma

    • Associated with familial adenomatous polyposis

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Symptoms and Signs

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  • • Jaundice

    • GI bleeding from ampullary tumor

    • Weight loss

    • Abdominal pain

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Laboratory Findings

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  • • Elevated serum bilirubin

    • Anemia

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Imaging Findings

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  • CT scan: Dilation of the biliary tree and pancreatic duct; also for staging

    Abdominal US: Dilated biliary tree and pancreatic duct

    ERCP: Dilation of the biliary and pancreatic ducts

    • In 75% of cases, the tumor is visible as an exophytic papillary lesion, an ulcerated tumor, or an infiltrating mass

    • In 25% of cases, there is no intraduodenal growth, and endoscopic sphincterotomy is necessary to display the tumor

    • An adequate biopsy specimen can usually be obtained from these lesions

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  • • If a tumor of the ampulla of Vater is suspected but not visualized on duodenoscopy, a sphincterotomy should be performed to inspect the intraluminal surface of the ampulla

    • A biopsy specimen of the tumor should be obtained to confirm an ampullary neoplasm and differentiate adenoma from adenocarcinoma

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Rule Out

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  • • Benign causes of biliary obstruction

    • Cholangiocarcinoma

    • Pancreatic adenocarcinoma

    • Duodenal adenoma or adenocarcinoma

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  • • Abdominal US to identify biliary and pancreatic duct dilatation

    • ERCP to identify lesion and perform biopsy

    • CT scan for staging

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When to Admit

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  • • Symptomatic, high-grade biliary obstruction not amenable to endoscopic treatment

    • Actively bleeding tumors

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  • Adenomas: Local excision or pancreaticoduodenectomy; if invasive cancer found after excision, pancreaticoduodenectomy

    Adenocarcinoma: Pancreaticoduodenectomy

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Surgery

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Indications

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  • • All ampullary adenomas and adenocarcinomas

    • Metastases to resectible peripancreatic lymph nodes is not a contraindication

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Contraindications

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  • • Distant metastases (hepatic): Endoscopic biliary drainage only

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Complications

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  • • Biliary and pancreatic duct obstruction

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Prognosis

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  • • < 1 year survival without resection

    • 5-year survival after resection of adenocarcinoma is 50%

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References

Bakaeen FG et al: What prognostic factors are important in duodenal adenocarcinoma? Arch Surg 2000;135:635.  [PubMed: 10843358]
Crucitti A et al: Ampullary carcinoma: prognostic significance of ploidy, cell-cycle analysis and proliferating cell ...

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