Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis

被引：8

作者：

Bass, Lee M. ^{[1
]}

Patil, Deepa ^{[2
]}

Rao, M. Sambasiva ^{[2
]}

Green, Richard M. ^{[3
]}

Whitington, Peter F. ^{[1
]}

机构：

[1] Northwestern Univ, Dept Pediat, Feinberg Sch Med, Chicago, IL 60611 USA

[2] Northwestern Univ, Dept Pathol, Feinberg Sch Med, Chicago, IL 60611 USA

[3] Northwestern Univ, Dept Med, Feinberg Sch Med, Chicago, IL 60611 USA

来源：

BMC GASTROENTEROLOGY | 2010年 / 10卷

关键词：

EXPORT PUMP DEFICIENCY; CANCER; MUTATIONS; LIVER; BILE; CHILDREN; CHOLANGIOCARCINOMA; FEATURES; ABCB11; TISSUE;

D O I：

10.1186/1471-230X-10-30

中图分类号：

R57 [消化系及腹部疾病];

学科分类号：

摘要：

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.

引用

页数：5

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