Papillary-cystic neoplasm of the pancreas

Papillary-cystic neoplasm of the pancreas is a rare tumor which has recently been described as a distinct, clinicopathological entity. Forty-four previously described cases from the literature and an additional 52 cases referred to in a brief abstract are reviewed. An additional case occurring in a 25-year-old woman is reported. This tumor occurs primarily in young women (mean age 23.3 years) and presents as a large abdominal mass (mean size 10.3 cm) in 63% of patients. Treatment is surgical resection of the involved portions of the pancreas and results of treatment are excellent following this form of therapy alone. The tumor appears to represent a low-grade malignancy, possibly of ductal cell origin. Only two patients with recurrences and two patients with metastatic disease have been reported. Surgeons should be aware of this curable pancreatic malignancy as they are most often responsible for the evaluation and treatment of the patient presenting with a large abdominal mass.