Solid and cystic papillary neoplasms of the pancreas: report of four cases.

BACKGROUND/AIMS: In this report we present four cases with solid and cystic papillary neoplasms (SCPN) of the pancreas, and discuss the histopathological and immunohistochemical findings with a review of the literature.
METHODS: The four cases reported here consisted of three women (ages 20-48, mean: 32) and one man (age: 58). The clinical diagnoses were confirmed with ultrasound (US) and computerized tomography (CT). The surgical management of the tumors included enucleation (1 patient), distal pancreatectomy with splenectomy (1) and distal pancreatectomy (2).
RESULTS: The tumors were large (mean diameter of the resected tumor was 15 cm), had cystic degenerations between solid areas, and were distributed in the body and the tail of the pancreas. The cystic spaces contained hemorrhagic, necrotic and thrombotic material. The immunohistochemical studies revealed that the four tumors were all positive for a1 antitrypsin and neuron specific enolase, and were all negative for chromogranin. Vimentin and synaptophysin were positive in three different cases. The follow-up of the patients has been uneventful for 2, 1, 7 and 12 years, respectively.
CONCLUSIONS: SCPN of the pancreas is an uncommon clinicopathologic entity with a relatively low grade malignant potential. The majority of the cases are young women. Fifty percent of the cases are asymptomatic, and the patients with symptoms generally suffer from an abdominal mass or abdominal pain. In spite of the characteristic macroscopic and microscopic aspects, the immunophenotypical view is nonspecific. Prognosis is excellent after complete surgical resection and recurrence is rarely seen. Metastatic spread is not expected and the tumor usually has a manner of local invasion. Acinar cell carcinoma, pancreatoblastoma and pancreatic endocrine tumor must be considered in the differential diagnosis.