Case Presentation: A 47-year-old male with a history of untreated ulcerative colitis (UC) presented to the emergency department with a two-week history of jaundice, malaise, fatigue, right upper quadrant pain, and tan-colored stools. Vital signs were normal. Physical exam was remarkable for epigastric tenderness, sclera icterus and jaundice. Relevant laboratory findings revealed total bilirubin of 116.28 µmol/L (direct: 88.92 µmol/L), alkaline phosphatase of 1382 IU/L, aspartate aminotransferase of 179 IU/L, alanine aminotransferase of 135 IU/L and normal lipase. The patient was admitted for the evaluation of obstructive jaundice. Initial workup consisted of extensive imaging. Magnetic resonance cholangiopancreatography (MRCP) revealed a poorly defined enhancing mass lesion centered around the gallbladder penetrating the liver parenchyma and measuring 6.2 x 4.8 cm. Other neighboring lesions on the liver that were concerning for metastasis from presumed gallbladder malignancy were present. Biliary ductal system occlusion by an infiltrating process was found at the level of the junction of right and left hepatic ducts and was accompanied by intrahepatic biliary ductal dilatation. Computed tomography (CT) of the chest with intravenous contrast revealed multiple small pulmonary nodules. The patient developed a fever and a percutaneous transhepatic cholangiography was performed with a drain placed for decompression. The procedure revealed a beaded display of the dilated left-sided intrahepatic biliary duct concerning for primary sclerosing cholangitis (PSC). There was a high-grade stricture on the left hepatic duct. Carbohydrate antigen 19-9 (CA 19-9) was 177 kU/L, carcinoembryonic antigen was 19.2 μg/L, and alpha-fetoprotein was normal at 2 µg/L. Liver enzymes decreased after the drain was placed. Ultrasound-guided right hepatic lobe biopsy showed extensive portal and lobular inflammation with the inability to exclude PSC. CT-guided biopsy of the gallbladder fossa mass confirmed hilar cholangiocarcinoma (CCA). This case did not meet criteria for liver transplantation, which consisted of large duct disease (< 3 cm) with no invasion into the liver. The patient was not a surgical resection candidate and was subsequently started on chemotherapy.

Discussion: PSC is a chronic cholestatic disease characterized by inflammation and fibrosis of the biliary tract, ultimately leading to cirrhosis and increased risk of cholangiocarcinoma. 2-7% of patients with UC may develop PSC. Our case depicts an unusual presentation of PSC and CCA at the same time and the similarities among both clinical presentations may cause clinicians to overlook the other. An MRCP demonstrating multifocal intra- and extrahepatic bile duct strictures with adjacent bile duct dilation can be sufficient for the diagnosis of PSC. The presence of a focal mass, bile duct wall thickening greater than 4 mm, and progressive biliary obstruction indicate CCA against the background of PSC.

Conclusions: Inflammatory and neoplastic disorders of the intrahepatic and extrahepatic biliary system may have similar clinical presentations and radiological features making differentiation difficult. Thereby, histopathological diagnosis is usually confirmatory. CCA usually presents during the seventh decade of life and prognosis is poor in non-surgical cases. Our patient was relatively younger and the simultaneous presence of PSC and CCA at diagnosis makes this case unusual and complex. Liver transplant may provide some benefit in select cases.

IMAGE 1: Percutaneous transhepatic cholangiography demonstrating “beads on a string” appearance of the biliary tract

IMAGE 2: MRCP revealing metastatic lesions on the liver