Treatment for ampullary cancer is usually with surgery to remove the cancer. For very small cancers, surgical tools can be passed down the throat, through the stomach, and into the small intestine to cut away the cancer. A larger cancer might require more extensive surgery to remove the cancer, the gallbladder, and portions of the bile duct, pancreas, and small intestine. Chemotherapy with powerful drugs and radiation with energy beams might also be options.

To diagnose ampullary cancer, the doctor passes a long thin tube equipped with a camera down the throat, through the stomach, and into the small intestine. The camera is used to look for signs of cancer in the ampulla of Vater, which is the area where ducts leading from the liver and pancreas connect to the small intestine. Special tools passed through the tube can collect a sample of cells for testing. Imaging tests like CT, MRI, and ultrasound might also be used.