Neuroendocrine tumors in children and young adults: rare or not so rare

Abstract

This review focuses on neuroendocrine tumors (NETs) that arise in the diffuse neuroendocrine system; these rare tumors can develop in any organ that has dispersed single endocrine cells, for example, the intestine, or in an organ that has clusters of endocrine cells, for example, pancreatic islets. Previously considered benign, NETs are now recognized to recur locally or metastasize to liver and bone if not completely excised early in their course of development. This article summarizes the epidemiology and reviews the diagnostic and therapeutic challenges of NETs in children and youth, noting especially those NETs that are more prevalent in young people than in older adults.