Post written by Sooraj Tejaswi, MD, MSPH, FASGE, from the University of California Davis School of Medicine, Sacramento, California, and Texas Digestive Disease Consultants, The Woodlands, Texas, USA.

A 63-year-old woman had incidentally noted abnormal liver test results with transaminitis (aspartate aminotransferase 104 U/L, alanine transaminase 233 U/L), elevated alkaline phosphatase (290 U/L), and mild hyperbilirubinemia (total bilirubin 1.7 mg/dL) and intra- and extrahepatic biliary dilation with filling defects on magnetic resonance imaging (MRI) with MRCP. She underwent ERCP.

A prominent ampulla and excessive bleeding were noted after needle-knife papillotomy. A papillary ampullary mass prolapsed on balloon sweep, raising suspicion for an intra-ampullary papillary-tubular neoplasm. Intraductal extension was noted on digital cholangioscopy. Biopsies showed low-grade dysplasia.

The patient chose surveillance, and progression to intramucosal adenocarcinoma was noted over a 9-month period. Extension into the pancreatic duct was viewed on EUS examination. Cancer-free margins were achieved with a Whipple resection, and the surgical pathology confirmed well-differentiated intra-ampullary invasive intestinal—type adenocarcinoma (T2N0M0). The patient was doing well 18 months after surgery.

Intra-ampullary papillary-tubular neoplasms are rare tumors with the best prognosis of all ampullary tumors with prompt surgical resection, despite them being bulkier than ampullary adenocarcinomas and ampullary-ductal carcinomas.

Being cognizant of the differential diagnoses of ampullary lesions will help with early diagnosis by means of appropriate work-up that includes ERCP, diligent ampullary examination, EUS examination of the distal common bile duct (CBD) and pancreatic duct to detect intraductal extension—which can be missed on cross-sectional imaging—and cholangioscopy to map intraductal extension into the bile duct. Early diagnosis and prompt surgical resection are associated with a good prognosis.

Other endoscopists can learn the following from this experience:

• Review radiographic images personally. In our case, MRI with MRCP showed filling defects in the distal CBD that were atypical of stones because of the noted papillary nature of the filling defects and ampullary mass.
• If there is an unusual amount of postsphincterotomy or postpapillotomy bleed, have a low threshold to suspect ampullary abnormalities such as tumors.
• Cholangioscopy can provide supportive information to help establish the diagnosis if characteristic features of papillary neoplasms are seen in the CBD, namely copious mucin, frond-like growths, and increased vascularity.
• Undertake early EUS to assess intraductal extension into the pancreatic and bile ducts and to examine regional lymph nodes.
• Refer to a pancreaticobiliary surgeon early in the disease process because the tumor has a good prognosis with pancreaticoduodenectomy in the absence of distant metastases.

Ampullary mass noted on balloon sweeps.

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