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Clinical manifestations and diagnosis of ampullary adenomas

INTRODUCTION

Benign neoplasms of the ampulla of Vater are rare, representing less than 10 percent of periampullary neoplasms, the majority of which are malignant tumors arising from the duodenum, ampulla, or pancreas [1,2]. The most common benign lesions are villous and tubulovillous adenomas; others include hemangiomas, leiomyomas, leiomyofibromas, lipomas, lymphangiomas, and neurogenic tumors [3,4].

Although classified as benign, ampullary adenomas have the potential to undergo malignant transformation to ampullary carcinomas, similar to the adenoma-to-carcinoma sequence that occurs elsewhere in the gastrointestinal tract [1,5-13]. As a result, their clinical significance extends beyond the need to treat any associated symptoms.

Ampullary adenomas can occur sporadically, or in the setting of familial polyposis syndromes such as familial adenomatous polyposis (FAP) and its related conditions. Approximately 40 to 100 percent of patients with ampullary adenomas in the setting of FAP have coexisting duodenal adenomatous polyps, which are frequently numerous and also have a malignant potential [14-18]. (See "Clinical features and diagnosis of familial adenomatous polyposis".)

The prevalence of ampullary adenomas has been estimated to be 0.04 to 0.12 percent in autopsy series [5,19,20]. However, they are being increasingly recognized with the extensive availability of flexible endoscopy and the widespread application of screening and surveillance programs for high-risk patients such as those with FAP [7,14,16,18,21].

This topic review will focus on the clinical manifestations and diagnosis of ampullary adenomas. (See "Treatment of ampullary adenomas".)
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