Case 194: Periampullary Hepatoid Adenocarcinoma with Duodenal Invasion

History A previously healthy 48-year-old man presented with progressive asthenia and fatigue of several weeks duration associated with sporadic episodes of diarrhea and a 22-kg weight loss in the previous 6 months. He was afebrile. Physical examination revealed jaundice and a palpable epigastric mass. He reported a 10-pack-year smoking history and moderate alcohol intake (30-40 g/d). The patient had no previous oncologic history, and he was seronegative for the human immunodeficiency virus, as well as for hepatitis B and C. Laboratory tests revealed a hemoglobin level of 6.3 g/dL (normal range, 13.8-18.0 g/dL) and elevated levels of serum liver enzymes (alanine transaminase level, 98 U/L [normal level, <60 U/L]; aspartate transaminase level, 121 U/L [normal level,,40 U/L]), gamma-glutamyl transpeptidase (2232 U/L [normal range, 0-51 U/L), total bilirubin (5.2 mg/dL [88.9 mu mol/L]; normal range, 0.1-1.2 mg/dL [1.71-20.52 mu mol/L]), and direct bilirubin (4.7 mg/dL [80.37 mu mol/L]; normal range, 0.1-0.4 mg/dL [1.71-6.84 mu mol/L]). Serum a-fetoprotein (AFP) level was elevated (15 300 ng/mL; normal level, <10 ng/mL). The remaining tumor marker levels-including carcinoembryonic antigen, CA-19-9 (carbohydrate antigen 19-9), CA-125 (carbohydrate antigen 125), and neuron-specific enolase-were normal. The initial imaging examination was abdominal ultrasonography (US). Endoscopic US was performed 3 days later and revealed an extrinsic compression of the duodenal bulb by a large solid tumor mass infiltrating the second and third portions of the duodenal arch, precluding the progression of the US probe. Magnetic resonance (MR) imaging was performed for further evaluation.