Abstract
Neuroendocrine tumors (NETs) of the gastrointestinal (GI) tract can be broadly classified as gastrointestinal NETs (GI-NETs) also referred to as previously and more commonly as carcinoid tumors and pancreatic NETs (PNETs). The NETs can be functional (secreting peptides and amines) or nonfunctional and are classified based on histology, mitotic counts, and Ki67 staining into low, intermediate, and high grade, that is, Grades 1, 2, and 3, respectively. In this chapter, we briefly review the physiology and biochemistry of the hormones resulting in clinical syndromes and then discuss the clinical presentation and diagnosis of the more common syndromes. Whilst we discuss the clinical syndromes such as PNETs and GI-NETs, it is important to emphasize that some of these tumors can emanate from both the pancreas and intestinal cells (e.g., gastrinomas). It appears that up to 25% are associated with inherited disorders including multiple endocrine neoplasia type 1 (MEN1), von Hippel–Lindau disease, neurofibromatosis 1 (NF-1), and tuberous sclerosis complex. It appears that the commonest type of NET is a nonfunctional PNET. In fact, loss of heterozygosity at the MEN1 locus on chromosome 11q13 is seen in 93% of sporadic PNETs.
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