Neurendocrine tumors (NETs) is a rare tumor with incidence about 2 cases per 100,000 persons but they represent a clinical challenge because of their varied presentations and initial studying images to locate the tumors may be inconclusive. Based on the characteristic of peptide and amine producing of those tumor cells, the intracellular markers of endocrine tissue, such as chromogranin A, synaptophysin, and neuron-specific enolase can be used in the diagnosis. NETs can be classified as carcinoid tumors or functional and non-functional NETs. While functional NETs and carcinoid tumors can be revealed by their dramatic clinical symptoms due to it over production of endogenous hormones or vasoactive substances, nonfunctional NETs are silent and therefore may be metastasized at the time of detection. This review will summarize the heritable tumor syndromes that related to NETs, the main clinical symptoms as well as the methods to diagnose NETs.
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