Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Neuroendocrine Tumors of the Pancreas

  • Manfred Schwab
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4033-2


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...


Neuroendocrine Tumor Vasoactive Intestinal Peptide Neuroendocrine Carcinoma Carcinoid Syndrome Islet Cell Tumor 
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See Also

  1. (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
  2. (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

Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.German Cancer Research Center (DKFZ)HeidelbergGermany