A man with repeated cholangitis

Abstract

A 67-year-old man, with a past medical history of remarkable left intrahepatic biliary stones and recurrent episodes of acute cholangitis, presented to our hospital with low-grade fever and epigastric dull aching followed by tea-colored urine for 1 week. Initial laboratory data were as follows: Hb 11.5 g/dL, WBC (white blood count) 17 700/mm3, CRP (C-Reactive Protein) 25.13 mg/dL, AST/ALT (aspartate transaminase/alanine transaminase) 39/45 IU/L, bilirubin (T/D) 7.5/4.6 mg/dL, alkaline phosphatase 596 IU/L, CEA (carcinoembryonic antigen) 1.92 ng/mL, AFP (Alpha-FetoProtein) 4.10 ng/mL, PSA (prostate specific antigen) 5.57 ng/mL, and CA 19-9 (Carbohydrate Antigen 19-9) 92.56 U/mL. Abdominal computed tomography demonstrated a new-onset mass lesion (10 cm in diameter) occupying the left lobe of liver. Dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) of the liver demonstrated a 10 cm liver mass with dilatation of adjacent IHDs (intrahepatic duct) in the left lobe (mainly in segment 4), indicating a malignant neoplasm (Figure 1). The preoperative diagnosis was ICC according to his clinical presentation and radiological images. He received an extended left hepatectomy after a thorough preoperative evaluation, including the ICG (Indocyanine green) test. The histopathological examination demonstrated intraductal papillary neoplasm of bile duct (IPNB) with low-grade dysplasia and marked chronic inflammation (Figure 2). His postoperative course was uneventful, and he was discharged 10 days after the operation. IPNB, formerly named biliary papillomatosis, is a rare biliary epithelial tumor with an exophytic nature exhibiting a papillary mass within the bile duct lumen and with a prominent intraductal growth pattern. It has been considered as a precursor lesion of invasive cholangiocarcinoma. The grade of intraepithelial neoplasia is defined as either low-intermediate, high, or invasive carcinoma according the World Health Organization's 2010 classification system.1 The clinical picture of IPNB consists of recurrent colicky abdominal pain and repeated episodes of acute cholangitis with fever and jaundice due to partial or intermittent biliary obstruction.2 Common radiological findings for IPNB are bile duct dilatation or intraductal masses, and liver solid tumors are rarely seen. It is difficult to distinguish IPNB from other inflammatory and neoplastic lesions based on radiological appearance alone, including DCE-MRI of liver.3 Patients with IPNB should be considered for treatment because of the high risk of recurrent cholangitis and malignant transformation. Resection is the treatment of choice when IPNB is localized according to preoperative imaging workup. The overall prognosis is better in patients with IPNB than ICC, and long-term survival is anticipated after curative resection.4 The author declares no conflict of interest.