Abstract
The ampulla of Vater is a crucial anatomical junction that regulates the flow of bile and pancreatic secretions into the duodenum. Despite the rarity of nonmalignant tumors of this region, they have significant clinical importance. Ampullary adenomas are the most common benign tumors in this area and are considered premalignant lesions with a substantial risk of malignant progression, requiring complete resection upon diagnosis. Ampullary neuroendocrine tumors and gangliocytic paragangliomas are relatively rare but may be associated with hormone production or local metastatic potential, highlighting the need for accurate diagnosis and individualized treatment strategies. Ampullary adenomyomas and other rare benign lesions generally have a benign course but can clinically and radiologically mimic a malignancy, often necessitating surgical resection for a definitive diagnosis. This review comprehensively summarizes the anatomy of the ampulla of Vater and the pathological features and clinical significance of nonmalignant ampullary tumors. Continued research and multidisciplinary collaboration are crucial for developing effective treatment strategies and improving patient outcomes in this complex field.