Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc
Journal website http://www.journalmc.org

Case Report

Volume 4, Number 10, October 2013, pages 654-659

Intraductal Papillary Neoplasm of the Bile Duct Arising From a Background of Autoimmune Hepatitis: A Case Report

Figures

Figure 1.
Figure 1. Liver imaging. (A) CT-angiography. The cystic tumor with solid masses in its cavity, showing faint arterial staining in the medial segment of the liver. (B) Intraductal ultrasonography (IDUS) of the choledochus. Two solid, irregular masses arise from the wall of the cystic tumor.
Figure 2.
Figure 2. Histology of the tumor. (A) Intraductal papillary structures of the epithelial tumor cells with a fibrous stroma. Magnification: × 40. (B) Adenocarcinoma components extend along the epithelium of the intrahepatic bile ducts, showing a carcinoma in situ. Magnification: × 400. Hematoxylin and Eosin staining.
Figure 3.
Figure 3. Immunohistochemical staining of the tumor showing a pancreatobiliary-type intraductal papillary neoplasm of the bile duct. The cytoplasmic expression of both MUC5A and MUC6 as mucin-related proteins is positive in the epithelial tumor cells. The cells show nuclear expression of Ki-67 and cytoplasmic expression of CA19-9, suggesting their malignant potential. Magnification: × 200.
Figure 4.
Figure 4. Histology of the background liver. (A) Interface hepatitis with plasma cell infiltration is compatible with the histology of autoimmune hepatitis. Magnification: × 400. (B) Proliferation of intrahepatic bile ducts is also demonstrated. Magnification: × 200. Hematoxylin and Eosin staining.

Table

Table 1. Laboratory Data on Admission
 
BiochemistryHematologyCoagulation
AST, aspartate transferase; ALT: alanine aminotransferase; LDH: lactate dehydrogenase; ALP: alkaline phosphatase; GGT: γ-glutamyl transpeptidase; BUN: blood urea nitrogen; IgM-HAAb: IgM-hepatitis A antibody; HBsAg/Ab: hepatitis B surface antigen/antibody; HBcAb: hepatitis B core antibody; HCV Ab: hepatitis C virus antibody; INR: International normarized ratio; AMA: anti-mitochondrial antibody; DUPAN-2: pancreatic cancer associated antigen.
  Total protein7.3 g/dLWhite blood cell5,910/µLINR1.13
  Albumin3.3 g/dLRed blood cell452 × 104/µLImmunology
  Total bilirubin8.8 mg/dLHemoglobin14.3 g/dLImmunoglobulin G2,200 mg/dL
  Direct bilirubin7.8 mg/dLHematocrit42.3%Immunoglobulin A521 mg/dL
  Indirect bilirubin1.0 mg/dLPlatelet22.0 × 104/µLImmunoglobulin M199 mg/dL
  AST271 U/LViral markeranti-nuclear antibody(+)160 ×
  ALT580 U/LIgM-HAAb(-)AMA(-)
  LDH439 U/LHBsAg(-)Tumor marker
  ALP173 U/LHBsAb(-)α-fetoprotein24 ng/mL
  GGT175 U/LHBcAb(-)carcinoembryonic antigen0.98 ng/mL
  BUN17 mg/dLHCV Ab(-)carbohydrate antigen 19-914.4 U/mL
  Creatinine0.8 mg/dLDUPAN-2470 U/mL