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A 54-Year-Old Man Comes to the Office Due to Increasing

question 126

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A 54-year-old man comes to the office due to increasing pain in the right upper quadrant, nausea, and unintentional weight loss.  Vitals signs are within normal limits.  BMI is 21 kg/m2.  The patient appears cachectic and there is scleral icterus.  The liver is palpable 3 cm below the right costal margin.  No ascites is present.  Laboratory studies reveal elevated bilirubin and alkaline phosphatase.  Contrast CT scan of the abdomen demonstrates moderately dilated right and left intrahepatic bile ducts, with a vague area of delayed enhancement at their convergence.  Biopsy of the lesion reveals columnar cells with hyperchromatic oval nuclei with prominent nucleoli arranged in glandular structures; mucin production with surrounding desmoplastic reaction is present.  Which of the following is the most likely diagnosis?


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