Neuroendocrine tumour of the pancreas

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Neuroendocrine tumour of the pancreas, also pancreatic neuroendocrine tumour, is a relatively uncommon tumour.

It may be abbreviated PanNET.[1]

Previously, it was referred to as pancreatic islet cell tumour or islet cell tumour; thes terms are now considered to be outdated.[1]

General

Classification

Based on peptide produced in the pancreatic islets:

  1. Glucagon from alpha cells (glucagonoma).
  2. Insulin from beta cells (insulinoma) - most common ~ 50% of islet cell tumours.
  3. Somatostatin from D cells (somatostatinoma).
  4. Pancreatic polypeptide from PP cells.

Others:

  1. Vasoactive intestinal peptide (VIPoma).
  2. Gastrin (gastrinoma).
    • May be seen in Zollinger-Ellison syndrome.
      • Triad: pancreatic gastrinoma, gastric acid hypersecretion, marked peptic ulcers in the small bowel.[4]

Gross

  • Usually in the head of the pancreas - 68% in one series,[5] and 50% in another series.[5]

Microscopic

Features:

  • Nests of cells.
  • Stippled chromatin.
  • +/-Hyaline globules.

DDx:

Images

www:

IHC

  • CK19 +ve -- should be done as a routine in pancreatic NETs; poor prognostic factor.[6]

Note:

  • CK19 should not be confused with CA19-9.

See also

References

  1. 1.0 1.1 Burns, WR.; Edil, BH. (Dec 2011). "Neuroendocrine Pancreatic Tumors: Guidelines for Management and Update.". Curr Treat Options Oncol. doi:10.1007/s11864-011-0172-2. PMID 22198808.
  2. Charlesworth, M.; Verbeke, CS.; Falk, GA.; Walsh, M.; Smith, AM.; Morris-Stiff, G. (Feb 2012). "Pancreatic Lesions in von Hippel-Lindau Disease? A Systematic Review and Meta-synthesis of the Literature.". J Gastrointest Surg. doi:10.1007/s11605-012-1847-0. PMID 22370733.
  3. Alexakis, N.; Connor, S.; Ghaneh, P.; Lombard, M.; Smart, HL.; Evans, J.; Hughes, M.; Garvey, CJ. et al. (2004). "Hereditary pancreatic endocrine tumours.". Pancreatology 4 (5): 417-33; discussion 434-5. doi:10.1159/000079616. PMID 15249710.
  4. Zollinger RM, Ellison EH (1955). "Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas". Ann. Surg. 142 (4): 709–23; discussion, 724–8. doi:10.1097/00000658-195510000-00015. PMC 1465210. PMID 13259432. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1465210/.
  5. 5.0 5.1 Oh, TG.; Chung, MJ.; Park, JY.; Bang, SM.; Park, SW.; Chung, JB.; Song, SY. (Sep 2012). "Prognostic factors and characteristics of pancreatic neuroendocrine tumors: single center experience.". Yonsei Med J 53 (5): 944-51. doi:10.3349/ymj.2012.53.5.944. PMC 3423842. PMID 22869477. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3423842/. Cite error: Invalid <ref> tag; name "pmid22869477" defined multiple times with different content
  6. Jain, R.; Fischer, S.; Serra, S.; Chetty, R. (Jan 2010). "The use of Cytokeratin 19 (CK19) immunohistochemistry in lesions of the pancreas, gastrointestinal tract, and liver.". Appl Immunohistochem Mol Morphol 18 (1): 9-15. doi:10.1097/PAI.0b013e3181ad36ea. PMID 19956064.