Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis.
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.


METHODS
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.
Bass
Lee M
LM
Northwestern University Feinberg School of Medicine, Department of Pediatrics, Chicago, Illinois, USA. lbass@childrensmemorial.org
Patil
Deepa
D
Rao
M Sambasiva
MS
Green
Richard M
RM
Whitington
Peter F
PF
D000230:Adenocarcinoma / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D000328:Adult; D002780:Cholestasis, Intrahepatic / Q000150:complications*; D005260:Female; D006801:Humans; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy