An 80-year-old man with a medical history of hypertension, hyperlipidemia, benign prostatic hyperplasia, and gastroesophageal reflux disease presented to the emergency department with a complaint of fever starting 8 hours ago. He endorsed poor oral intake and abdominal pain in the right upper quadrant that started 2 weeks ago associated with postprandial pain. He noticed yellowing of his skin and eyes, dark-colored urine 5 days earlier, and a 10-pound weight loss over the past few weeks. The patient denied any sick contacts, travel history, or previous gastrointestinal issues. He had mild nausea and vomiting, denied smoking, alcohol, or drug use.

On evaluation in the emergency department, he was hemodynamically stable and afebrile. The patient was in no visible distress, overweight habitus, and appropriately alert and oriented. There was apparent bilateral scleral icterus with jaundiced and dry skin. The abdomen was nondistended with normal bowel sounds, and tenderness to palpation in the epigastric region was appreciated with no guarding or rigidity. The laboratory results were significant for elevated white blood cell count of 26.8 K/mcL, alkaline phosphatase of 494 IU/L, aspartate transaminase of 134 IU/L, alanine transaminase of 198 IU/L, and an elevated total bilirubin of 4.6 mg/dL with an elevated direct bilirubin of 2.4 mg/dL. Blood cultures were drawn, and he was started on intravenous piperacillin-tazobactam. A computed tomography (CT) of the abdomen and pelvis with intravenous and oral contrast showed no hepatic lesions, no intrahepatic biliary dilatation, a dilated common bile duct (CBD) to 1.3 cm with an abrupt caliber change and concern for wall thickening, and normal-appearing pancreas with no pancreatic ductal dilatation (Figure 1). Owing to the leukocytosis, subjective fevers, jaundice, and bile duct thickening with contrast enhancement, there was concern for acute cholangitis. An endoscopic ultrasound was completed which found sludge in the gallbladder but no gallbladder mass, and extrahepatic bile duct dilatation at the level of the confluence and common hepatic duct measuring up to 12 mm with abrupt caliber change in the mid bile duct at the level of the cystic duct takeoff. Bile duct wall thickening was noted. There was no regional lymphadenopathy, and the pancreatic duct and left lobe of the liver were unremarkable. A subsequent endoscopic retrograde cholangiopancreatography (ERCP) showed moderate-to-severe stenosis extending approximately 1.5 cm in the mid CBD (Figure 2). This was located in the proximity of the cystic duct takeoff. A 10-mm wire-guided biliary sphincterotomy was performed. Wire-guided SpyDS cholangioscopy was performed and demonstrated mucosal nodularity and neovascularization at the level of the stricture. No stones were visualized. Under direct visualization, SpyMax forceps biopsies were obtained of the stricture site and wire-guided brushings of the stricture for cytology. A 10 × 80 mm fully covered metal biliary stent was deployed with prompt drainage of contrast and purulent bilious fluid. Morphology by ERCP and cholangioscopy was concerning for neoplasm and less likely for Mirizzi syndrome. Given the findings on imaging and endoscopic intervention, the stricture was extrahepatic and extrapancreatic in nature. Subsequent laboratory results showed an elevated CA 19-9 of 55 units/mL. Immunohistochemistry completed for pathology on ERCP sampling of the CBD was used to determine that this is a neuroendocrine carcinoma. The atypical cells coexpressed cytokeratin cocktail AE1/AE3, synaptophysin, chromogranin, and CD56, which are specific for neuroendocrine tumors. Ki-67 proliferation rate was > 20% (range 20%–40%), which is suggestive of grade 3 of 3 tumor. He was taken to the operating room for resection of the common hepatic duct, CBD, cystic duct, hepatic hilar lymph node dissection, cholecystectomy, pancreaticoduodenectomy, and hepaticojejunostomy. The patient tolerated the procedure well and was managed in the surgical intensive care unit postoperatively. Final pathology from the operation revealed large-cell neuroendocrine carcinoma of the CBD invading into the periductal soft tissue to a depth of 4 mm with 18 negative lymph nodes. Two months after surgical resection, the patient completed a gallium 68 DOTA-TATE positron emission tomography-CT scan that showed no abnormal radiotracer accumulation. He had no evidence of metastatic disease. At a 6-month follow-up with hematology oncology, he was feeling well despite mild increase in frequency of bowel movements. Repeat CT imaging at 6 months showed no abnormal findings suspicious for recurrence.