A rare case of periampullary carcinoma with ectopic ending of Vater’s ampulla

Abstract

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 mo. Magnetic resonance imaging and magnetic resonance cholangiopancreatography (MRCP) showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm × 5 cm × 5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater's ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.

Ectopic Opening of the Common Bile Duct Accompanied by Choledochocele and Pancreas Divisum

“…Some previous reports described the presence of the major papilla in the second portion of the duodenum (1, 6, 7). In other reports, the pancreatic duct was visualized in fewer than half of the total cases (2)(3)(4)(5)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), which suggests the possibility of another orifice joining with the pancreatic duct. Moreover, from the viewpoint of the embryonic process, the presence of the major papilla is consistent with Boyden's theory (30), as described above.…”

“…Previous reports have suggested that the incidence of PD in autopsy cases is about 9% (21,22). To our knowledge, this is the first report of an ectopic opening of the CBD into the duodenal bulb accompanied by choledochocele and PD (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Choledochocele concomitant with PD is also rare, with only three case reports having been published (25)(26)(27); therefore, our case is extremely rare.…”

“…Furthermore, the dorsal pancreatic bud develops on the opposite side of the hepatic diverticulum. At five to seven weeks of gestation, the ventral pancreatic bud and bile ducts rotate from right to left, although the duodenum rotates clockwise, and the ventral and dorsal pancreatic buds fuse together (9,21,28,29). PD occurs because of a failure of the dorsal and ventral pancreatic ducts to fuse during this embryogenetic process (21).…”

“…Ectopic opening of the common bile duct (CBD) is a rare congenital biliary anomaly, with the CBD emptying into anomalous sites such as the third or fourth portion of the duodenum, stomach, or duodenal bulb (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Choledochocele is also a biliary anomaly, with cystic dilatation of the intraduodenal segment of the CBD (18)(19)(20).…”

Tubulovillous adenoma of the duodenal papilla: radiological-endoscopic and anatomopathological correlation in the surgical proposal

“…Fluctuating jaundice and biliary colic may progressively appear with the growth of these tumors. Less frequently, pancreatitis and upper gastrointestinal bleeding have been reported [1][2][3] . In addition to diagnosis, complementary imaging tests with increasingly improved resolution can improve individualized surgical planning and discussion.…”