Carcinoma of the Ampulla of Vater Clinical Presentation

Updated: Dec 31, 2015
  • Author: Nafisa K Kuwajerwala, MD; Chief Editor: N Joseph Espat, MD, MS, FACS  more...
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Presentation

History

Jaundice is the presenting symptom in 73% of resected patients and in 80% of unresected patients (71% overall) in the 28-year experience published by Talamini et al. [8]

Jaundice may intermittently wax and wane because of central necrosis and sloughing or pressure opening of a minimally obstructed duct. Patients with malignant tumors commonly have jaundice and larger tumors. [9]

Other features are as follows:

  • Progressive weight loss is the second most common symptom in 61%.
  • Abdominal pain and back pain were present in 46%. The abdominal pain usually is dull, aching midepigastric pain or right hypochondriac pain.
  • Back pain may be a sign of advanced stage.
  • Pruritus associated with jaundice in 13-38%.
  • Dyspepsia and vomiting may be present if compromise of the duodenal lumen leads to gastric outlet obstruction.
  • Loss of appetite may be noted.
  • Diarrhea may occur with this tumor due to the absence of lipase within the gut related to pancreatic duct obstruction.
  • Gastrointestinal bleeding occurs in 5% of resected and in 22% of unresected patients.
  • Acute pancreatitis was significantly more common in unresected lesions (28%) compared with resected lesions (2%). [8]
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Physical

Physical examination findings may include the following:

  • The Courvoisier sign, painless jaundice associated with a palpable gallbladder, may be present. Unlike that due to a neoplasm, obstructive jaundice due to a stone causes scarring of the gallbladder, precluding its distension.
  • Fever can occur in the setting of ascending cholangitis.
  • Hepatomegaly can occur.
  • Rarely, patients present with features of acute pancreatitis or migratory thrombophlebitis.
  • Palpable fixed epigastric masses or supraclavicular nodes are signs of advanced disease and inoperability.
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Causes

The etiology of the disease is poorly understood.

  • Patients with familial adenomatous polyposis (FAP) have an increased risk of both benign and malignant ampullary tumors. [10]
  • As many as 50-90% of patients with FAP develop duodenal adenomas, predominantly concentrated on or around the major papilla. [11]
  • K- ras mutations may be a factor. [12]
  • Microsatellite instability is associated with a better prognosis.
  • Chromosome 17p and 18q loss of heterozygosity are associated with ampullary carcinoma. [13]
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