Papillary cystic neoplasm of the pancreas.

BACKGROUND: Papillary cystic neoplasm of the pancreas is a rare disorder that occurs most commonly in young women. It has a low potential for malignancy, and the prognosis following resection is favourable. CASE OUTLINE: An 18-year-old white girl presented with a palpable mass in the right hypochondrium on physical examination associated with epigastric pain, nausea and vomiting, but no fever. Upper gastro-intestinal endoscopy revealed extrinsic compression of the posterior wall of the antrum and duodenal bulb with no mucosal lesion. Computed tomography (CT) scan and then laparotomy revealed a large tumour adjacent to the hepatic hilum and originating from the head of pancreas. Pancreatoduodenectomy was performed, and a diagnosis of papillary cystic neoplasm of the pancreas was made.There was no evidence of recurrence after 6 years of follow-up. DISCUSSION: A radical surgical approach is justified for papillary cystic neoplasm of the pancreas because of its biological behaviour, local aggressiveness and low incidence of metastases.