2 型进行性家族性肝内胆汁淤积症中的胰腺腺癌。
Pancreatic adenocarcinoma in type 2 progressive familial intrahepatic cholestasis.
机构信息
Northwestern University Feinberg School of Medicine, Department of Pediatrics, Chicago, Illinois, USA.
出版信息
BMC Gastroenterol. 2010 Mar 13;10:30. doi: 10.1186/1471-230X-10-30.
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.
背景
BSEP 疾病是由 ABCB11 基因突变引起的，该基因编码胆汁盐输出泵（BSEP）。BSEP 疾病与肝胆癌的风险增加有关。
病例介绍
一位 36 岁的 BSEP 疾病女性在 36 岁时患上了胰腺腺癌。她在 18 岁时接受了胆道分流术。腹部 CT 扫描发现胰腺内有一个 1.7x1.3 厘米的肿块。在胰腺颈部和近体部发现一个 2 厘米的肿块病变。病理显示为 2-3 级腺癌，侵犯胰周脂肪。
结论
临床医生应意识到 BSEP 疾病患者中可能发生胰腺腺癌。
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