A 40-year-old woman complains of having severe back pain for about 3 months and recurrent fever. Her past medical history is significant for ulcerative colitis. On physical examination, the patient is thin and jaundiced. The liver edge descends 1 cm below the right costal margin and is nontender. Laboratory studies show normal serum levels of AST and ALT but elevated serum levels of alkaline phosphatase (420 U/L). Endoscopic retrograde cholangiopancreatography demonstrates a beaded appearance of the extrahepatic biliary tree. Which of the following diseases is a late complication of this patient's condition?
Adenocarcinoma of the gallbladder
Cholangiocarcinoma
Hepatic adenoma
Hepatic angiosarcoma
Primary sclerosing cholangitis (PSC) is characterized by inflammation and obliterative fibrosis of intrahepatic and extrahepatic bile ducts, with dilation of preserved segments. Approximately 70% of patients with PSC have longstanding ulcerative colitis, although the prevalence of PSC in such patients is only 4%. PSC tends to occur in the third through fifth decades of life, with a significant male predominance (2:1). The clinicopathologic findings are complemented by a characteristic radiographic appearance of a beaded biliary tree, representing sporadic strictures. Cholangiocarcinoma is a late complication of PSC. The other choices are not complications of PSC.Diagnosis: Primary sclerosing cholangitis
Liver & Biliary Tract