Papillary cystic neoplasm of the pancreas in children: report of three cases

Abstract

Papillary cystic neoplasm of the pancreas is very rare in children. There were only 35 pediatric cases reported in the literature. We herein three children who had papillary cystic neoplasm of pancreas. They were female teenagers, and were pathologically diagnosed. The major presenting symptoms were abdominal pain and abdominal mass. Serum tumor markers of these patients showed normal results. A CT scan of these patients showed that this tumor was of pancreatic origin. These 3 tumors were localized to head, body, and tail, respectively. The mean maximal diameter of these tumors was 11.3 +/- 3 cm. Sonography and CT examination showed that the tumor was a heterogeneous mass with solid and cystic components. Angiography of this tumor showed a hypervascular mass with blood supply mainly from pancreatic branch of splenic artery. They all underwent tumor resection. All tumors contained some degree of internal hemorrhage or cystic degeneration and all were well encapsulated. Histologically, tumor cells generally showed solid and pseudopapillary growth around the fibrovascular stalks. No metastasis, mortality or recurrence was noted during follow-ups. In conclusion, CT scan helps to make a prospective diagnosis of papillary cystic neoplasm of pancreas. Our study confirmed that a papillary neoplasm of the pancreas is a low-grade malignant tumor. Surgical resection of the tumor is the mainstay of effective management.