Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis

Autor: Green Richard M, Rao M Sambasiva, Patil Deepa, Bass Lee M, Whitington Peter F
Jazyk: angličtina
Rok vydání: 2010
Předmět:
Zdroj: BMC Gastroenterology, Vol 10, Iss 1, p 30 (2010)
Druh dokumentu: article
ISSN: 1471-230X
DOI: 10.1186/1471-230X-10-30
Popis: 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 × 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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