Case Presentation: A 64-year-old woman with a history of cholecystectomy and chronic intermittent abdominal pain presented with acute onset of right upper quadrant pain and vomiting. On admission, she was afebrile and nontoxic appearing with mild epigastric tenderness. Labs showed an elevated AST/ALT, total bilirubin, and alkaline phosphatase. Abdominal ultrasound showed a dilated common bile duct (CBD) and no obstructing gallstone. MRCP demonstrated two stones in the CBD measuring 0.7 and 1.1 cm with intra and extrahepatic biliary dilatation. No suspicious lesions were seen. Bilirubin improved the following day and she requested to be discharged prior to ERCP with plans to return. Three days later patient re-presented with a repeat episode of acute abdominal pain with exam and labs consistent with cholangitis. Antibiotics were started and ERCP was performed showing stones in the CBD and a 25 mm fungating mass in the major papilla with pus emerging from the major papilla. A CBD stent was placed and pathology of a small portion of the mass revealed tubulovillous adenoma. GI had continued concern for adenocarcinoma given location and appearance of the mass and requested a general surgery consultation. General surgery consultation recommended CT abdomen with contrast to confirm the ampullary mass which demonstrated biliary stent and no ampullary mass. Patient was discharged on oral ciprofloxacin with plans for follow-up with general surgery for consideration of a Whipple Procedure.

Discussion: Due to a slow rate of growth, and vague symptomatology late in the disease process, biliary tract adenocarcinomas are often diagnosed in advanced stages with a poor prognosis. The majority of cases are discovered in the setting of evaluation for painless jaundice from biliary obstruction. When more closely reviewing the patient’s history, she had been complaining of more typical symptoms of abdominal pain for years, but had never developed jaundice. Ductal dilatation is seen in 75% of cases, with cholangitis being a less common presentation. An ERCP is the preferred imaging modality as it permits biopsy and placement of a stent for biliary decompression, if necessary. MRCP is a highly reliable means to detect biliary tree tumors, but does not allow for intervention. Other imaging modalities to consider include CT abdomen with contrast and endoscopic ultrasound.

Conclusions: Cholangitis is an uncommon presentation of a rare disease. In this case, presenting with cholangitis allowed for early detection and treatment of a likely ampullary carcinoma compared to the oftentimes later presentation of obstructive jaundice. This may indicate an improved clinical outcome. This case also clarifies the role of different imaging modalities in detecting and defining ampullary masses. As in this case, imaging of an ampullary mass requires a multi-modal approach to imaging for an accurate diagnosis.