Neuroendocrine Tumors of the Pancreas
Pancreatic NETs, which have traditionally been referred to as islet cell tumors, are accompanied by hormonal activity in about 50–60 %. Distinct clinical syndromes are related to the inappropriate secretion of insulin (hyperinsulinemic hypoglycemia), glucagon (glucagonoma syndrome), gastrin (Zollinger-Ellison syndrome, ZES), somatostatin (hypersomatostatinemia), vasoactive intestinal peptide (watery diarrhea syndrome), growth hormone releasing hormone (acromegaly), adrenocorticotropic hormone (Cushing Syndrome), serotonin (carcinoid syndrome), and others. The term used to designate various subtypes reflects the specific hypersecretory syndromes (insulinoma, gastrinoma). Nonfunctioning NETs may contain certain hormones but are silent (nonsyndromic).
Pancratic NETs with a diameter of ≤1 cm, a <2 % Ki-67 index, and absence of angioinvasion are generally benign. When the size is >2 cm and the Ki-67 index <2 %, NETs may have a benign or low-grade malignant behavior. Tumors with a...
KeywordsNeuroendocrine Tumor Vasoactive Intestinal Peptide Neuroendocrine Carcinoma Carcinoid Syndrome Islet Cell Tumor
- (2012) Ki-67. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 1943. doi: 10.1007/978-3-642-16483-5_3213Google Scholar
- (2012) Zollinger–Ellison syndrome. In: Schwab M (ed) Encyclopedia of cancer, 3rd edn. Springer, Berlin/Heidelberg, p 3980. doi: 10.1007/978-3-642-16483-5_6303Google Scholar