Cancer of the major duodenal papilla (papilla or ampulla of Vater) develops at the junction of the bile ducts with the duodenum. The main risk factor is familial adenomatous polyposis (FAP).
Symptoms: As in distal cholangiocarcinoma (located in peripheral extrahepatic bile ducts), but jaundice occurs earlier (therefore diagnosis is made at a relatively early stage); duodenal obstruction may occur.
Diagnosis: Duodenoscopy or endoscopic retrograde cholangiopancreatography (ERCP) with specimen collection by biopsy or brushing.
Treatment: Pancreatoduodenectomy; at an early stage, resection of the ampulla of Vater is possible (endoscopic or open ampullectomy). In the case of an unresectable lesion, endoscopic papillotomy and biliary drainage through a prosthesis or percutaneous drainage is performed to alleviate cholestasis. Bypass anastomosis may be necessary if duodenal obstruction occurs.