AGA Clinical Practice Update on Management of Ampullary Neoplasms: Expert Review

Although ampullary neoplasia is a rare entity and represents 0.6%-0.8% of gastrointestinal cancers, the incidence among young adults has risen over the past 20 years. Most ampullary tumors are sporadic, but a few genetic syndromes substantially increase the risk of ampullary neoplasia. Compared with other neoplastic pancreaticobiliary entities, there is a relative lack of guidance on best practices for management of ampullary lesions. Ampullary cancer is associated with a 5-year survival rate ranging from 20% to 75%, depending on the stage of diagnosis. Ensuring high-quality endoscopic assessment and treatment with complete resection will decrease recurrence risk and identify patients at an earlier stage.

This expert review was commissioned and approved by the American Gastroenterological Association Institute Clinical Practice Updates Committee and the American Gastroenterological Association Governing Board to provide timely guidance on a topic of high clinical importance to the American Gastroenterological Association membership and underwent internal peer review by the Clinical Practice Updates Committee and external peer review through standard procedures of Clinical Gastroenterology and Hepatology. These Best Practice Advice statements were drawn from a review of the published literature and from expert opinion. Because systematic reviews were not performed, these Best Practice Advice statements do not carry formal ratings regarding the quality of evidence or strength of the presented considerations. BEST PRACTICE ADVICE STATEMENTS BEST PRACTICE ADVICE 1: Assessment for ampullary adenomas is best conducted using a side-viewing duodenoscope, because ampulla visualization and access for biopsies with a standard forward-viewing gastroscope is limited. Furthermore, use of a duodenoscope allows for a more careful biopsy, avoiding the pancreatic orifice to minimize postprocedure pancreatitis risk. BEST PRACTICE ADVICE 2: A significant proportion (20%-40%) of ampullary adenomas harbor malignancy; therefore, at least 6 biopsies should be obtained to assess histopathology, especially from ulcerated or indurated areas. BEST PRACTICE ADVICE 3: Endoscopic ultrasound assessment is valuable for staging and determination of the extent (if any) of intraductal extension and should be considered for each patient with an ampullary adenoma that is potentially amenable to endoscopic resection, barring lesions smaller than 1 cm with no worrisome clinical or endoscopic features. BEST PRACTICE ADVICE 4: Endoscopic characteristics of an ampullary adenoma that suggest it is benign and render it most amenable to endoscopic papillectomy include: well-defined margins, size less than 4 to 5 cm, a nonfriable lesion, and a lesion that is soft to endoscopic probing and with intraductal extension limited to 1 cm or less. BEST PRACTICE ADVICE 5: For ampullary adenomas with significant (>1 cm) intraductal extension, consultation for consideration of surgical resection is suggested. Lesions with minimal (<1 cm) intraductal involvement may be amenable to endoscopic treatment with close surveillance. BEST PRACTICE ADVICE 6: Three definitive contraindications to endoscopic papillectomy are: metastatic spread, invasive cancer that extends beyond the mucosal layer, and with >1cm intraductal extension in a surgically fit patient. Relative contraindications or factors that warrant further consideration include lesions over 5 cm in size, early-stage/T1 cancers and concerns surrounding patients with limited follow-up adherence. BEST PRACTICE ADVICE 7: In cases where endoscopic resection is not feasible, and biliary obstruction resulting in significant jaundice or when cholangitis is present, biliary drainage with or without sphincterotomy and self-expandable metal stent placement may be considered at the discretion of the surgeon. BEST PRACTICE ADVICE 8: Avoid prepapillectomy biliary and pancreatic sphincterotomies to minimize the risk of incomplete histopathologic assessment due to electrocautery artifact and bleeding prior to resection. BEST PRACTICE ADVICE 9: En bloc snare resection should be performed when feasible. Submucosal injection is generally not required but may be beneficial for laterally spreading components of the lesion along the duodenal wall. BEST PRACTICE ADVICE 10: Prophylactic pancreatic duct stenting, rectal nonsteroidal anti-inflammatory drugs, and high-volume hydration with lactated Ringer's solution should be used to minimize postpapillectomy pancreatitis. BEST PRACTICE ADVICE 11: Standard hemostasis techniques, such as epinephrine injection, electrocoagulation, use of hemoclips, argon plasma coagulation, and hemostatic sprays, may all be carefully employed for treating bleeding from endoscopic papillectomy. BEST PRACTICE ADVICE 12: Following endoscopic papillectomy, surveillance should begin at 3 months, with side-viewing duodenoscopy and biopsy of the resection scar and any abnormal area. If there is no residual adenoma, surveillance should be repeated at 6 months and 12 months, and then annually for at least 5 years. Surveillance beyond 5 years should be individualized based on recurrence risk, comorbidity, and life expectancy. In familial adenomatous polyposis, surveillance intervals should be individualized according to ampullary, gastric, and duodenal disease burden. BEST PRACTICE ADVICE 13: For adenomas >3 cm resected piecemeal or those with positive margins, consider adjunct thermal ablation and ductography. Perform this follow-up every 2-3 months until eradication is confirmed via biopsy. BEST PRACTICE ADVICE 14: There are some reports of recurrence or cancer developing more than 5 years after resection; however, these cases are rare. Due to the limited number of such reports, the decision to continue endoscopic surveillance beyond 5 years should be individualized based on the overall health and life expectancy of the patient. If high-risk features are present, long-term endoscopic monitoring is required. BEST PRACTICE ADVICE 15: Patients with high-grade dysplasia, incomplete resection, or suspected malignancy should be discussed by a multidisciplinary team that includes gastroenterologists, surgeons, and oncologists.