Pancreatic intraductal papillary mucinous neoplasms: a paradigmatic case. A case report and review of the literature

Pancreatic intraductal papillary mucinous neoplasms constitute an increasingly frequent clinical entity. The definition and clinical behavior of these tumours are still a subject of debate. As a consequence, their clinical management also presents controversial aspects ranging from follow-up to the advisability or otherwise of an aggressive surgical approach, even in the case of small non-malignant lesions. In 2002 we observed a patient affected by a large pancreatic mass with the endoscopic and radiological features of an intraductal papillary mucinous tumour. Over a 20-year clinical history the patient had been considered and treated as affected by chronic pancreatitis. In spite of the tumour size and possible vascular infiltration, surgical exploration was considered. Total pancreatectomy was performed and final histology revealed a non-invasive papillary mucinous carcinoma of the pancreas. Twenty-six months after surgical resection the patient is alive and free of disease. In the present paper we re-assess the clinical history of this patient and review the most recent literature on such tumours.