Papillary cystic tumor of the pancreas. Report of six cases and a review of the literature

Papillary cystic tumor of the pancreas is a rare, but unique, clinico-pathological entity which has recently received increased attention. Six cases which illustrate the typical clinical features and indolent nature of the tumor are presented. All patients were young women, average age of 15 years, except for a 55-year-old woman who had noted a slow-growing mass in the left upper abdomen for at least 26 years. Clinical characteristics include pain and mass in most patients, in whom the finding is usually incidental. The tumors ranged in size from 10 to 16 cm (average size, 11.3 cm) and were located in the tail (5 patients) and in the head (1 patient) of the pancreas. All six patients underwent resection, which include distal pancreatectomy (three patients), distal pancreatectomy and splenectomy (two patients) and pancreaticoduodenectomy (one patient). There were no distant metastases or local invasions. No evidence of recurrence or metastasis was noted during the follow-up period. Grossly, the tumor is usually ovoid or multi-loculated in shape and well-encapsulated. Microscopically, the tumor is characterized by solid sheets and papillary growth around a thin fibrovascular core. Papillary cystic tumor is considered to be a low-grade malignancy, and the prognosis tends to be excellent. It is potentially curable by surgical resection, so an aggressive surgical treatment is mandatory.