An annular pancreas associated with carcinoma of the papilla of Vater: report of a case

Abstract

An annular pancreas is an uncommon congenital anomaly that usually presents early in childhood. Malignancy in the setting of an annular pancreas is unusual. We herein report a case of annular pancreas with carcinoma of the papilla of Vater. A 59-year-old man presented with epigastric discomfort and was referred to us after gastroduodenal endoscopy showed a tumor of the papilla of Vater. Preoperative imaging showed the pancreatic parenchyma encircling the descending duodenum and a tumor at the papilla of Vater. A pancreaticoduodenectomy was performed for the annular pancreas and the ampullary tumor. Histological examination confirmed a complete annular pancreas and carcinoma in situ of the papilla of Vater. We also provide a review of the reported cases of an annular pancreas with periampullary neoplasms and discuss the clinical characteristics of this anomaly.

Carcinoma of the Ampulla of Vater Associated with Annular Pancreas

“…AP was first described by Tiedemann in 1818 and was termed Bannular pancreas^in 1862 by Ecker. The first successful surgery for an annular pancreas, a gastrojejunostomy, was performed in 1905 by Vidal [3].…”

“…A review of the 106 cases by Yogi et al [6] found nine (8.5 %) having a pancreaticobiliary malignancy (five ampullary carcinomas, two pancreatic carcinomas, and two cholangiocarcinomas). Previously, only seven cases of ampullary carcinoma with AP have been reported in the English literature [3].…”

“…Popular theories explaining the pathogenesis include adhesion of the right ventral bud to the anterior duodenal wall and failure of the left ventral bud to involute [2]. AP is usually asymptomatic but is also known to present at times with duodenal obstruction, pancreatitis, peptic ulceration, and rarely obstructive jaundice with coexistent periampullary cancer [3].…”

Pancreaticoduodenectomy for pancreas carcinoma occurring in the annular pancreas: report of a case

“…Among patients undergoing endoscopic retrograde cholangiopancreatography (ERCP), approximately one in 1000 examined cases was found to have the annular pancreas [ 3 , 12 ]. In these cases, ERCP may not always be successful, due to technical difficulty in some cases, particularly in cases with the duodenal ulcer and/or stenosis of the descending part of the duodenum [ 13 ]. In most cases, multi-slice CT shows the circumferential pancreatic parenchyma around the descending part of the duodenum [ 13 , 14 ]; however, in some cases, this imaging method may lead to misinterpretation as a thickening of the duodenum [ 15 ].…”

“…In these cases, ERCP may not always be successful, due to technical difficulty in some cases, particularly in cases with the duodenal ulcer and/or stenosis of the descending part of the duodenum [ 13 ]. In most cases, multi-slice CT shows the circumferential pancreatic parenchyma around the descending part of the duodenum [ 13 , 14 ]; however, in some cases, this imaging method may lead to misinterpretation as a thickening of the duodenum [ 15 ]. With this in mind, magnetic resonance imaging (MRI) and MRCP are useful for understanding anomalies observed in the pancreatic anatomy [ 13 , 15 , 16 ].…”