Gastroenteropancreatic neuroendocrine (carcinoid) tumors in children

Abstract

Neuroendocrine tumors (NETs) (previously termed carcinoids) are slow-growing tumors of the neuroendocrine system. They can occur anywhere within the body but are most commonly found in the midgut. This review is therefore confined to a discussion of gastroenteropancreatic NETS (GEP-NETS). GEP-NETS may be asymptomatic and are found incidentally (eg, during appendicectomy) or can present with symptoms attributable to either the site of the primary tumor or the secretion of serotonin and other substances from metastatic carcinoid disease (carcinoid syndrome). Symptoms of carcinoid syndrome include facial flushing, diarrhea, wheezing, colicky abdominal pain, and edema. Surgical resection offers the only curative treatment for neuroendocrine tumors, although peptide hormone analogues can be used to control carcinoid symptoms. Guidelines exist to determine when further surgical resection is required when NETs (carcinoids) are found incidentally during appendicectomy. A multi-disciplinary approach is essential for the management of all children with these rare and challenging tumors.

Keywords: Appendix; Carcinoid; GEP-NET; Neuroendocrine tumor; Pancreas.