Fish-mouth appearance of the ampulla of Vater

Iosif Beintaris^a, Demetrios Polymeros, Silvia Krivan^b, Konstantinos Triantafyllou^a

Attikon University General Hospital, Medical School, University of Athens, Greece

^aHepatogastroenterology Unit, 2nd Department of Internal Medicine and Research Institute, Attikon University General Hospital, Medical School, University of Athens, (I. Beintaris, D. Polymeros, K. Triantafyllou); ^bThird Surgical Department, Attikon University General Hospital, Medical School, University of Athens (S. Krivan), Greece

Conflict of Interest: None

Correspondence to: Konstantinos Triantafyllou, Hepatogastroenterology Unit, 2nd Department of Internal Medicine and Research Institute, Attikon University General Hospital, Medical School, University of Athens, Rimini 1, 12462 Greece, Tel.: +30 210 5832090, Fax: +30 210 5326422, e-mail: ktriant@med.uoa.gr

Received 12 October 2012; accepted 29 October 2012

Cystic tumors of the pancreas account for less than 10% of pancreatic neoplasms. Intraductal papillary mucinous carcinomas (IPMNs) account for 21-33% of these tumors. IPMNs represent papillary neoplasms within the main pancreatic duct and/or side branches showing mucin hypersecretion that often leads to duct dilation and chronic obstructive pancreatitis [1].

A 55-year-old patient presented with a history of epigastric pain beginning 5 years ago. During this period the patient had experienced an episode of acute pancreatitis, shortly after which a computed tomography (CT) of the abdomen revealed a pancreatic pseudocyst.

An upper endoscopy using a side view endoscope was performed, revealing a patulous ampulla of Vater with extruding mucus (Fig. 1), the fish-mouth sign, practically pathognomonic for an IPMN.

IMPNs represent potentially malignant lesions, occurring equally in both genders. Abdominal pain and weight loss are the most common complaints. A history of recurrent pancreatitis is given by 20% of patients, and acute pancreatitis is found in 25% at presentation. Evaluation involves CT or MRI of the abdomen,endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasonography (EUS). Typically, dilation of the pancreatic duct with or without an associated cystic mass is demonstrated. EUS with FNA (fine needle aspiration) provides important information such as the degree of dysplasia, cell-type, extent of disease and malignant potential. Treatment of IPMNs frequently requires pancreatic resection, which successfully relieves symptoms and prevents progression to invasive carcinoma. Prognosis after resection of IPMN is excellent, with five-year disease specific survival of 75% or better [2,3].

References

1. Longnecker DS, Adler G, Hruban RH, et al. Intraductal papillary-mucinous neoplasms of the pancreas. In: Hamilton SR, Aaltonen LA (editors): World Health Organization classification of tumours. Pathology and genetics of tumours of the digestive system, Lyon, France: IARC Press; 2000:237-240.

2. Salvia R, Fernandez-del Castillo C, Bassi C, et al. Main-duct intraductal papillary mucinous neoplasms of the pancreas: clinical predictors of malignancy and long-term survival following resection. Ann Surg 2004;239:678-685.

3. D’Angelica M, Brennan MF, Suriawinata AA, et al. Intraductal papillary mucinous neoplasms of the pancreas: An analysis of clinicopathologic features and outcome. Ann Surg 2004;239:400-408.