Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis

Bass, L. M.; Patil, D.; Rao, M. S.; Green, R. M.; Whitington, P. F.

BMC Gastroenterol. 2010 Mar 17; 10:30

Abstract

ABSTRACT: BACKGROUND: BSEP disease results from mutations in ABCB11, which encodes the bile salt export pump (BSEP). BSEP disease is associated with an increased risk of hepatobiliary cancer. CASE PRESENTATION: A 36 year old woman with BSEP disease developed pancreatic adenocarcinoma at age 36. She had been treated with a biliary diversion at age 18. A 1.7 x 1.3 cm mass was detected in the pancreas on abdominal CT scan. A 2 cm mass lesion was found at the neck and proximal body of the pancreas. Pathology demonstrated a grade 2-3 adenocarcinoma with invasion into the peripancreatic fat. CONCLUSIONS: Clinicians should be aware of the possibility of pancreatic adenocarcinoma in patients with BSEP disease.

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