Ampulla of Vater Tumors

• • 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

Epidemiology

• • 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

Symptoms and Signs

• • Jaundice

  • GI bleeding from ampullary tumor

  • Weight loss

  • Abdominal pain

Laboratory Findings

• • Elevated serum bilirubin

  • Anemia

Imaging Findings

• • 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

• • 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

Rule Out

• • Benign causes of biliary obstruction

  • Cholangiocarcinoma

  • Pancreatic adenocarcinoma

  • Duodenal adenoma or adenocarcinoma

• • Abdominal US to identify biliary and pancreatic duct dilatation

  • ERCP to identify lesion and perform biopsy

  • CT scan for staging

When to Admit

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

  • Actively bleeding tumors

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

  • Adenocarcinoma: Pancreaticoduodenectomy

Surgery

Indications

• • All ampullary adenomas and adenocarcinomas

  • Metastases to resectible peripancreatic lymph nodes is not a contraindication

Contraindications

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

Complications

• • Biliary and pancreatic duct obstruction

Prognosis

• • < 1 year survival without resection

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

References