Primary biliary cirrhosis with cancer of the ampulla of Vater

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A test for the detection of antimitochondrial antibodies has led many physicians to accept the diagnosis of primary biliary cirrhosis without laparotomy; however, the coexistence of biliary tract malignancy in primary biliary cirrhosis is a definite Possibility and has been reported on one occasion.1 We report a case of carcinoma of the ampulla of Vater occurring with primary biliary cirrhosis, and discuss the value of the antimitochondrial antibody as well as the potential role of endoscopic retrograde cholangiopancreatography (ERCP) in establishing a correct diagnosis.

Case report

A 57-year-old white woman was referred to the Cleveland Clinic on September 18, 1970, because of jaundice and diarrhea of 8 to 9 months’ duration. She described her stools as gray, bulky, and foul smelling. Other complaints were mild right upper quadrant abdominal discomfort, generalized bone pain, and a 3.6-kg weight loss. There was no history of pruritus, alcohol ingestion, exposure to toxins or drugs, or previous liver or biliary tract disease. On physical examination, the patient was thin and emaciated. She had scleral icterus, multiple spider angioma on face and neck, but no xanthelasma or xanthomas. The liver span was 30 cm and the spleen was palpable 2 cm below the left costal margin. There was no ascites or ankle edema. The physical examination was otherwise normal.

The results of the initial laboratory tests are listed in Tables 1 and 2. Antimitochondrial antibody was positive at greater than 1:160 serum dilution. Smooth muscle antibody and antinuclear factor were negative.

Barium enema and upper. . .



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