Papillary cystic neoplasm of the pancreas: A curable pancreatic tumor

Pancreatic neoplasms are common and a leading cause of death from malignant disease. Their aggressiveness as well as the poor results of treatment has led to a somewhat nihilistic attitude in their surgical management with an emphasis on palliation rather than cure. Papillary cystic neoplasm of the pancreas, although rare, is frequently confused with more malignant tumors which are less successfully treated by pancreatic resection. This neoplasm arises from ductular epithelium, usually occurs in young women, and often remains asymptomatic until it becomes manifest as an abdominal mass or by a complication, e.g., rupture, hemorrhage, or secondary infection. Because of lack of familiarity with the tumor as well as its unusual presentation, the diagnosis is infrequently made at the initial operation. Our experience with 3 young females with papillary cystic neoplasms of the pancreas is reported. Included is a review of the literature indicating the unique characteristics of the tumor. Although locally invasive, these tumors appear to have limited malignant potential and rarely metastasize. An aggressive surgical approach seems fully justified.