Pancreatic neoplasms in childhood and adolescence.

Pancreatic neoplasms are rare in children and adolescents; thus, our understanding of these tumors is still quite limited. We retrospectively reviewed clinical features and outcomes of all patients below 18 years of age with pancreatic neoplasms who were treated at Asan Medical Center between December 1994 and March 2010. Thirty-two patients were identified. Abdominal pain was the most common symptom. The median duration of diagnostic delay was 21 days. Nineteen patients were diagnosed with solid pseudopapillary tumors, 3 with lymphomas, 2 with pancreatoblastomas, 2 with poorly differentiated carcinomas, 2 with acinar cell cancers, 2 with endocrine tumors, 1 with peripheral (primitive) neuroectodermal tumor, and 1 with hemangioendothelioma. Gross complete resection of the primary tumor was achieved in 24 patients (75%), and 8 patients (25%) received chemotherapy. At a median follow-up of 34 months, the 5-year overall survival rate was 92.0 ± 5.5%. On multivariate analysis, histologic type was the only factor significantly predictive of survival (P=0.009). Patients with poorly differentiated carcinoma showed the worst survival probability. In cases of solid pseudopapillary tumors, surgical resection was generally curative and the prognosis was excellent. Patients with other malignant tumors, however, may require therapeutic strategies other than surgery.