Neuroendocrine Tumors

Laurent Brunaud, MD ■ Hans-Dietrich Roeher, MD ■ Dietmar Simon, MD

Neuroendocrine tumors (carcinoid tumors) are unusual, rare tumors that can be localized in all organs originating from the endoderm. They are derived from neoplastic proliferation of cells of the diffuse neuroendocrine system. These cells have been called enterochromaffin or Kulchitsky cells; they are ubiquitous throughout the gastrointestinal tract, urogenital tract, and bronchial epithelium and are considered to be the largest endocrine organ of the human body.13 The neuroendocrine system has been described as a diffuse network of nerve and endocrine cells with a common phenotype characterized by the simultaneous expression of general protein markers and hormonal products specific to each neuroendocrine cell.4 The incidence of neuroendocrine tumors is 1 per 100,000 per year. The most frequent sites are the small intestine (35%), the appendix (33%), the rectum (14%), and the bronchial system (9%). However, a marked increase in the percentage of lung-bronchial tumors and a decrease in the percentage of appendiceal neuroendocrine tumors have been reported.5 In organs that rarely develop malignant tumors, neuroendocrine tumors become the most frequent tumors; thus, 34% of all small intestinal cancers and 77% of all appendiceal tumors are neuroendocrine tumors. In organs that develop tumors more frequently (e.g., the colon, stomach, lung, and breast), neuroendocrine tumors make up only 1% of the tumors. The overall prevalence of neuroendocrine tumors is estimated at 0.5%.6

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