Pancreatic Neoplasms in Children

Abstract

Pancreatic neoplasms in children are very uncommon, representing less than 1 % of all solid tumors. Because of their rarity, our understanding of their natural history is limited. Pancreatic neoplasms are divided into epithelial and non-epithelial categories. Epithelial tumors are those with a cell line that resembles the lining of the pancreatic ducts, the lining of the pancreatic acini or the cells that form the islets of Langerhans, which can be functional or clinically silent. Non-epithelial tumors are those that arise from tissue of mesenchymal or ectodermal origin, which are extremely rare. In general, pancreatic neoplasms in children have an overall better prognosis than in adults, although some tumors in children are very aggressive, unresectable at diagnosis and have a poor survival rate. Among all different types of pancreatic neoplasms in children, pancreatoblastoma (PBT) and solid-pseudopapillary tumor (SPPT) are the most common ones in the first and second decade of life, respectively. Complete surgical resection is the key in the treatment of all pancreatic neoplasms in children.