Difference between revisions of "Neuroendocrine tumour of the pancreas"
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Previously, it was referred to as '''pancreatic islet cell tumour''' or '''islet cell tumour'''; thes terms are now considered to be outdated.<ref name=pmid22198808>{{Cite journal | last1 = Burns | first1 = WR. | last2 = Edil | first2 = BH. | title = Neuroendocrine Pancreatic Tumors: Guidelines for Management and Update. | journal = Curr Treat Options Oncol | volume = | issue = | pages = | month = Dec | year = 2011 | doi = 10.1007/s11864-011-0172-2 | PMID = 22198808 }}</ref> | Previously, it was referred to as '''pancreatic islet cell tumour''' or '''islet cell tumour'''; thes terms are now considered to be outdated.<ref name=pmid22198808>{{Cite journal | last1 = Burns | first1 = WR. | last2 = Edil | first2 = BH. | title = Neuroendocrine Pancreatic Tumors: Guidelines for Management and Update. | journal = Curr Treat Options Oncol | volume = | issue = | pages = | month = Dec | year = 2011 | doi = 10.1007/s11864-011-0172-2 | PMID = 22198808 }}</ref> | ||
Neuroendocrine tumours in general are dealt with in the ''[[neuroendocrine tumours]]'' article. | |||
==General== | ==General== | ||
*Rare. | *Rare ~ 1% of pancreatic tumours.<ref>{{Ref GLP|496}}</ref> | ||
*Presentation depends on subtype, e.g. for ''insulinoma'' the typical presentation is hypoglycemia. | *Presentation depends on subtype, e.g. for ''insulinoma'' the typical presentation is hypoglycemia. | ||
*May be part of a syndrome: | *May be part of a syndrome: | ||
| Line 68: | Line 70: | ||
==See also== | ==See also== | ||
*[[Pancreas]]. | *[[Pancreas]]. | ||
*[[Neuroendocrine tumours]]. | |||
==References== | ==References== | ||
Revision as of 14:54, 4 March 2014
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it was referred to as pancreatic islet cell tumour or islet cell tumour; thes terms are now considered to be outdated.[1]
Neuroendocrine tumours in general are dealt with in the neuroendocrine tumours article.
General
- Rare ~ 1% of pancreatic tumours.[2]
- Presentation depends on subtype, e.g. for insulinoma the typical presentation is hypoglycemia.
- May be part of a syndrome:
Classification
Based on peptide produced in the pancreatic islets:
- Glucagon from alpha cells (glucagonoma).
- Insulin from beta cells (insulinoma) - most common ~ 50% of islet cell tumours.
- Somatostatin from D cells (somatostatinoma).
- Pancreatic polypeptide from PP cells.
Others:
- Vasoactive intestinal peptide (VIPoma).
- Gastrin (gastrinoma).
- May be seen in Zollinger-Ellison syndrome.
- Triad: pancreatic gastrinoma, gastric acid hypersecretion, marked peptic ulcers in the small bowel.[5]
- May be seen in Zollinger-Ellison syndrome.
Gross
Microscopic
Features:
- Nests of cells.
- Stippled chromatin.
- +/-Hyaline globules.
DDx:
Images
www:
- Islet cell tumour (upmc.edu).
- Pancreatic NET with features of SPT (upmc.edu).
- Pancreatic NET - another case (upmc.edu).
IHC
- CK19 +ve -- should be done as a routine in pancreatic NETs; poor prognostic factor.[7]
Note:
- CK19 should not be confused with CA19-9.
Sign out
TAIL OF PANCREAS AND SPLEEN, DISTAL PANCREATECTOMY AND SPLENECTOMY: - CYSTIC PANCREATIC NEUROENDOCRINE TUMOUR. - UNREMARKABLE SURROUNDING PANCREAS WITH FAT. - SPLEEN WITHIN NORMAL LIMITS. - FOUR BENIGN LYMPH NODES. - NEGATIVE FOR MALIGNANCY.
Micro
The sections show pancreas with a large well-circumscribed tumour that centrally has a large cyst-like cavity. The tumour cells have moderate pale grey cytoplasm and round nuclei with salt and pepper chromatin. The tumour cells are arranged in cords and nests. No cholesterol clefts are readily apparent. No hyaline globules are identified. No papillary structures are apparent.
No necrosis is identified. No degenerative changes are apparent. Mitotic activity is seen focally. There are 2 mitoses per 10 high power fields, were one high power field has an area of 0.2376 mm*mm.
See also
References
- ↑ 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.
- ↑ Iacobuzio-Donahue, Christine A.; Montgomery, Elizabeth A. (2005). Gastrointestinal and Liver Pathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 496. ISBN 978-0443066573.
- ↑ 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.
- ↑ 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.
- ↑ 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/.
- ↑ 6.0 6.1 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 - ↑ 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.