Pancreatic tumors in childhood most commonly present with a palpable mass or with pain [
3]. Jaundice occurs much less commonly than in adults [
3]. Tumors of the pancreas can arise from exocrine or endocrine cells. The following tumor types are described in children and adolescents: ductal adenocarcinoma, acinar cell carcinoma, pancreatoblastoma, solid pseudopapillary tumor, and pancreatic endocrine neoplasms (benign and malignant) [
4]. In addition, there are reported cases of other tumor types, which either arise within the mass of the pancreas from other non-pancreatic cell types or immediately adjacent to it or involve the gland secondarily. Classic ductal adenocarcinoma of the pancreas, the common type in adults, is extremely rare in childhood. Most cases are in older literature. To examine the incidence trends and outcomes for children with pancreatic malignancies, the Surveillance, Epidemiology, and End Results Registry (1973 to 2004) was examined for pediatric patients with pancreatic malignancies (up to 19 years of age) [
5]. Malignant pancreatic neoplasms were identified in 58 patients. Women outnumbered men 1.9:1 (38 versus 20) for an age population-adjusted incidence of 0.021 and 0.015 per 100,000. Ductal adenocarcinoma, was identified in seven patients. A 90-year (1918 to 2007), single institution, retrospective review of all patients with neoplastic pancreatic masses was performed and published recently [
6]. Eighteen patients were identified with seven distinct histopathological subtypes. The most common were gastroenteropancreatic neuroendocrine, solid pseudopapillary, and acinar tumors. There were 6 benign and 12 malignant tumors. Surgery remains the keystone of treatment for pancreatic tumors in the pediatric age group, as in adults [
7]. Long-term disease-free survival in childhood pancreatic malignancies is achievable with complete surgical resection [
6]. There is no proven role for chemotherapy or radiation [
4]. The information available in the literature, particularly with regard to the role of chemotherapy and radiation, is anecdotal [
4]. These modalities should be reserved in unresectable and metastatic disease. Finally, it appears that ductal adenocarcinoma has a worse prognosis. According to a recent study, there was a significant difference in tumor type 15-year survival with ductal adenocarcinoma having the worst (23%) and solid cystic tumor the best (100%) [
5].