Carcinoid tumor of the papilla of Vater

Carcinoid tumor of the papilla of Vater is an extremly rare entity. Only 73 cases have been reported in the world literature to date and only 1 case in Hungary. This tumor differes clinically and prognostically from other carcinoid tumors of the gastrointestinal tract having a more agressive behaviour. The clinical feature is determinated by the expansion and infiltrative nature of the tumor. Endoscopic retrograde cholangiography (ERC) and endoscopic biopsy (EB) are the most accurate diagnostic methods, while endoscopic ultrasonography (EUS) has an utmost importancy in determinating the surgical strategy. Depending on the tumor size and the grade of invasion of other structures the surgical treatment can be local excision or radical resection. The authors present a case of a 67-year-old female patient who was admitted because of obstructive jaundice. On ERC the papilla was prominent but covered with normal mucosa. The main bile duct was markedly dilated with slight dilation of intrahepatic branches. Endoscopic sphyncterotomy was done and multiple biopsy specimen were taken. Hystology proved carcinoid tumor. Having detected local invasion by EUS a pylorus preserving pancreatoduodenectomy was performed. One year after the operation there is no recurrence of the tumor.