Intraductal Hepatocellular Carcinoma Without Evidence of Hepatic Parenchymal Involvement

Abstract

Cases of extrahepatic, intraductal hepatocellular carcinoma (HCC) without evidence of hepatic parenchymal lesions are exceedingly rare. Furthermore, symptoms of obstructive jaundice seldom herald the diagnosis of HCC. Given our patient's clinical presentation, extrahepatic cholangiocarcinoma was the initial presumed diagnosis, but further workup confirmed HCC. Our patient was a 69 year old Caucasian male with a history of cirrhosis secondary to alcohol abuse with multiple medical comorbidities. He presented to an emergency room with complaints of weight loss, abdominal cramping, generalized pruritus, acholic stools, and dark urine. On physical exam, he was noted to be jaundiced with scleral icterus. Exam was otherwise benign, including abdominal examination. Blood work was significant for an aspartate aminotransferase of 163 unit/L, alanine aminotransferase of 96 unit/L, alkaline phosphatase of 343 unit/L, total bilirubin of 17.4 mg/dL, international normalized ratio of 1.2, albumin of 3.0 gm/dL, alpha-fetoprotein (AFP) of 4.7 ng/mL, cancer antigen 19-9 (CA 19-9) of 57 unit/mL, and carcinoembryonic antigen of 4.1 ng/mL. Other laboratory values from a complete blood count and a compreshensive metabolic panel were within normal limits. A CT abdomen/pelvis and subsequent MRI revealed intrahepatic and extrahepatic biliary dilatation as well as a 1.5 cm mass resulting in a common hepatic duct obstruction concerning for extrahepatic hilar cholangiocarcinoma. There was no evidence of local or distant metastases, and no evidence of hepatic parenchymal involvement. Tissue diagnosis was pursued with ERCP. Bile duct brushings as well as a bile duct biopsy were consistent with HCC. He was evaluated by both transplant hepatology and surgical oncology, but was considered a poor surgical candidate because of medical comorbidities. Palliation was provided by stent placement and stereotactic body radiotherapy. Our patient's clinical presentation with obstructive jaundice, in the setting of a normal AFP, elevated CA19-9, and imaging with an extrahepatic, intraductal lesion without evidence of hepatic parenchymal involvement were initially concerning for cholangiocarcinoma. Further workup with a biopsy of the intraductal lesion, however, proved the diagnosis to be HCC. This case presentation calls into question the diagnostic yield of serum studies, tumor markers, and radiologic findings for the diagnosis of HCC.