Insulinoma pathophysiology: Difference between revisions

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==Genetics==
==Genetics==
*Insulinoma is transmitted in [mode of genetic transmission] pattern.
*Insulinoma is transmitted in Autosomal Dominant pattern when it is associated with MEN 1 syndrome
*Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
*Genes involved in the pathogenesis of insulinoma include ''MEN1''gene. Loss of heterozygosity of ''MEN1'' gene takes place on chromosome 11q13<ref name="pmid20146582">{{cite journal |vauthors=Shin JJ, Gorden P, Libutti SK |title=Insulinoma: pathophysiology, localization and management |journal=Future Oncol |volume=6 |issue=2 |pages=229–37 |year=2010 |pmid=20146582 |pmc=3498768 |doi=10.2217/fon.09.165 |url=}}</ref>
*The development of [disease name] is the result of multiple genetic mutations.
 
==Associated Conditions==
==Associated Conditions==
* Other pancreatic neuroendocrine tumors like [[gastrinoma]], [[glucagonoma]], [[VIPoma]], [[somatostatinoma]]
* Other pancreatic neuroendocrine tumors like [[gastrinoma]], [[glucagonoma]], [[VIPoma]], [[somatostatinoma]]

Revision as of 17:34, 17 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]Parminder Dhingra, M.D. [3]

Overview

Insulinoma may occur as part of other genetic syndromes such as multiple endocrine neoplasia type 1 and von Hippel-Lindau syndrome. On microscopic histopathological analysis, solid or gyriform patterns, usually without glands are characteristic findings of insulinoma.

Genetics

Microscopic Pathology

On microscopic histopathological analysis characteristic findings of insulinoma are:

  • Solid or gyriform patterns, usually without glands


Overview

  • It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
  • [Pathogen name] is usually transmitted via the [transmission route] route to the human host.
  • Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
  • [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
  • The progression to [disease name] usually involves the [molecular pathway].
  • The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

Pathogenesis

  • Insulinoma is a rare benign pancreatic neuroendocrine tumor that arises from B islet cells, which are cells that are normally involved in the production of insulin. Few insulinomas can also produce other hormones such as Serotonin, gastrin, ACTH, glucagon, and somatostatin [2]
  • They are usually small(90%), sporadic(90%), solitary(90%) and benign(90%) tumors.
  • It usually occur s pordically but 10% are found to be associated with MEN 1 syndrome.[3]Those associated with the MEN1 syndrome are usually malignant and higher recurrence rate(21% at 10 and 20 years) than in those without MEN 1 (5% at 10 and 7% at 20 years). [4].
  • It is thought that insulinoma is mediated by mTOR/P70S6K signaling pathway. Inhibitors of mTOR(rapamycin) or dual PI3K/mTOR(NVP-BEZ2235)thus have become new drugs for treating insulinoma. An oral mTOR inhibitor, Everolimus, make better glycemic control in people having insulinoma.[5] The pathway will give more possibilities for medical treatment.[6]


  • The progression to hypoglycemia is actually because of decreased glucose synthesis rather than increased use due to the direct effect of insulin on the liver.[8]

Genetics

  • Insulinoma is transmitted in Autosomal Dominant pattern when it is associated with MEN 1 syndrome
  • Genes involved in the pathogenesis of insulinoma include MEN1gene. Loss of heterozygosity of MEN1 gene takes place on chromosome 11q13[9]

Associated Conditions

Gross Pathology

  • On gross pathology insulinomas have a red brown appearance, are usually small and solitary tumors. Although there is a case report of a large(9cm), pedunculated and weighing more than 100g.[10]
Insulinoma- Red brown appearance. By Edward Alabraba et al. - Pancreatic insulinoma co-existing with gastric GIST in the absence of neurofibromatosis-1. World Journal of Surgical Oncology 2009, 7:18doi:10.1186/1477-7819-7-18, CC BY 2.0, https://commons.wikimedia.org/w/index.php?curid=6686376
  • Almost all insulinomas are present throughout the pancreas and extrapancreatic ones causing hypoglycemia are rare(<2%)[11]

Microscopic Pathology

  • On microscopic histopathological analysis, [feature2], and [feature3] are characteristic findings of insulinoma.
  • Histopathology of pancreatic endocrine tumor (insulinoma). H&E stain[12]

    Histopathology of pancreatic endocrine tumor (insulinoma). H&E stain[12]

  • Histopathology of pancreatic endocrine tumor (insulinoma)[12]

    Histopathology of pancreatic endocrine tumor (insulinoma)[12]

  • Histopathology of pancreatic endocrine tumor (insulinoma). Chromogranin A immunostain[12]

    Histopathology of pancreatic endocrine tumor (insulinoma). Chromogranin A immunostain[12]

  • Histopathology of pancreatic endocrine tumor (insulinoma). Insulin immunostain[12]

    Histopathology of pancreatic endocrine tumor (insulinoma). Insulin immunostain[12]

References

  1. ↑ Murray PD, McKenzie DT, Swain SL, Kagnoff MF (1987). "Interleukin 5 and interleukin 4 produced by Peyer's patch T cells selectively enhance immunoglobulin A expression". J Immunol. 139 (8): 2669–74. PMID 3498768.
  2. ↑ AlJadir, Saadi (2015). "Insulinoma: Literature's Review (Part 1)". Endocrinology&Metabolism International Journal. 2 (3). doi:10.15406/emij.2015.02.00025. ISSN 2473-0815.
  3. ↑ Callender GG, Rich TA, Perrier ND (2008). "Multiple endocrine neoplasia syndromes". Surg Clin North Am. 88 (4): 863–95, viii. doi:10.1016/j.suc.2008.05.001. PMID 18672144.
  4. ↑ 4.0 4.1 Service FJ, McMahon MM, O'Brien PC, Ballard DJ (1991). "Functioning insulinoma--incidence, recurrence, and long-term survival of patients: a 60-year study". Mayo Clin Proc. 66 (7): 711–9. PMID 1677058.
  5. ↑ Kulke MH, Bergsland EK, Yao JC (2009). "Glycemic control in patients with insulinoma treated with everolimus". N Engl J Med. 360 (2): 195–7. doi:10.1056/NEJMc0806740. PMID 19129539.
  6. ↑ Zhan HX, Cong L, Zhao YP, Zhang TP, Chen G, Zhou L; et al. (2012). "Activated mTOR/P70S6K signaling pathway is involved in insulinoma tumorigenesis". J Surg Oncol. 106 (8): 972–80. doi:10.1002/jso.23176. PMID 22711648.
  7. ↑ Cao, Yanan; Gao, Zhibo; Li, Lin; Jiang, Xiuli; Shan, Aijing; Cai, Jie; Peng, Ying; Li, Yanli; Jiang, Xiaohua; Huang, Xuanlin; Wang, Jiaqian; Wei, Qing; Qin, Guijun; Zhao, Jiajun; Jin, Xiaolong; Liu, Li; Li, Yingrui; Wang, Weiqing; Wang, Jun; Ning, Guang (2013). "Whole exome sequencing of insulinoma reveals recurrent T372R mutations in YY1". Nature Communications. 4. doi:10.1038/ncomms3810. ISSN 2041-1723.
  8. ↑ Rizza, R. A.; Haymond, M. W.; Verdonk, C. A.; Mandarino, L. J.; Miles, J. M.; Service, F. J.; Gerich, J. E. (1981). "Pathogenesis of Hypoglycemia in Insulinoma Patients: Suppression of Hepatic Glucose Production by Insulin". Diabetes. 30 (5): 377–381. doi:10.2337/diab.30.5.377. ISSN 0012-1797.
  9. ↑ Shin JJ, Gorden P, Libutti SK (2010). "Insulinoma: pathophysiology, localization and management". Future Oncol. 6 (2): 229–37. doi:10.2217/fon.09.165. PMC 3498768. PMID 20146582.
  10. ↑ Mittendorf EA, Liu YC, McHenry CR (2005). "Giant insulinoma: case report and review of the literature". J Clin Endocrinol Metab. 90 (1): 575–80. doi:10.1210/jc.2004-0825. PMID 15522939.
  11. ↑ Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K (2013). "Diagnosis and management of insulinoma". World J. Gastroenterol. 19 (6): 829–37. doi:10.3748/wjg.v19.i6.829. PMC 3574879. PMID 23430217.
  12. ↑ 12.0 12.1 12.2 12.3 Neuroendocrine tumour of the pancreas. Libre Pathology. http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_pancreas


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