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Pancreatic insulinoma

MedGen UID:
43907
Concept ID:
C0021670
Neoplastic Process
Synonym: Insulinoma
SNOMED CT: Insulinoma (302822000); Beta cell adenoma (302822000)
 
HPO: HP:0012197
Monarch Initiative: MONDO:0024677

Definition

A type of tumor of the pancreatic beta cells that secretes excess insulin and can result in hypoglycemia. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic insulinoma
Follow this link to review classifications for Pancreatic insulinoma in Orphanet.

Conditions with this feature

Multiple endocrine neoplasia, type 1
MedGen UID:
9957
Concept ID:
C0025267
Neoplastic Process
Multiple endocrine neoplasia type 1 (MEN1) includes varying combinations of more than 20 endocrine and non-endocrine tumors. Endocrine tumors become evident either by overproduction of hormones by the tumor or by growth of the tumor itself. Parathyroid tumors are the most common MEN1-associated endocrinopathy; onset in 90% of individuals is between ages 20 and 25 years with hypercalcemia evident by age 50 years; hypercalcemia causes lethargy, depression, confusion, anorexia, constipation, nausea, vomiting, diuresis, dehydration, hypercalciuria, kidney stones, increased bone resorption/fracture risk, hypertension, and shortened QT interval. Pituitary tumors include prolactinoma (the most common), which manifests as oligomenorrhea/amenorrhea and galactorrhea in females and sexual dysfunction in males. Well-differentiated endocrine tumors of the gastro-entero-pancreatic (GEP) tract can manifest as Zollinger-Ellison syndrome (gastrinoma); hypoglycemia (insulinoma); hyperglycemia, anorexia, glossitis, anemia, diarrhea, venous thrombosis, and skin rash (glucagonoma); and watery diarrhea, hypokalemia, and achlorhydria syndrome (vasoactive intestinal peptide [VIP]-secreting tumor). Carcinoid tumors are non-hormone-secreting and can manifest as a large mass after age 50 years. Adrenocortical tumors can be associated with primary hypercortisolism or hyperaldosteronism. Non-endocrine tumors include facial angiofibromas, collagenomas, lipomas, meningiomas, ependymomas, and leiomyomas.
Islet cell adenomatosis
MedGen UID:
293643
Concept ID:
C1578917
Neoplastic Process
Insulinomatosis and diabetes mellitus syndrome is an autosomal dominant disorder in which affected individuals within a family present with either hyperinsulinemic hypoglycemia secondary to pancreatic neuroendocrine tumors, or a noninsulin-dependent form of diabetes mellitus. A few affected individuals show only impaired glucose tolerance. Some patients also exhibit congenital cataract and/or congenital glaucoma (Iacovazzo et al., 2018).
Insulinoma tumor suppressor gene locus
MedGen UID:
335576
Concept ID:
C1847015
Finding

Professional guidelines

PubMed

Crinò SF, Napoleon B, Facciorusso A, Lakhtakia S, Borbath I, Caillol F, Do-Cong Pham K, Rizzatti G, Forti E, Palazzo L, Belle A, Vilmann P, van Laethem JL, Mohamadnejad M, Godat S, Hindryckx P, Benson A, Tacelli M, De Nucci G, Binda C, Kovacevic B, Jacob H, Partelli S, Falconi M, Salvia R, Landoni L; ERASING study collaborators, Larghi A
Clin Gastroenterol Hepatol 2023 Oct;21(11):2834-2843.e2. Epub 2023 Mar 4 doi: 10.1016/j.cgh.2023.02.022. PMID: 36871765
Mosquera-Klinger G, Carvajal JJ
Rev Esp Enferm Dig 2021 Jan;113(1):48-51. doi: 10.17235/reed.2020.7109/2020. PMID: 33226258
Pellicano R, Fagoonee S, Altruda F, Bruno M, Saracco GM, De Angelis C
Minerva Endocrinol 2016 Dec;41(4):490-8. PMID: 27600643

Recent clinical studies

Etiology

Damaskos C, Dimitroulis D, Garmpi A, Antoniou EA, Kouraklis G, Psilopatis I, Mavri M, Diamantis E, Marinos G, Kyriakos G, Farmaki P, Patsouras A, Kontzoglou K, Garmpis N
In Vivo 2023 Nov-Dec;37(6):2402-2408. doi: 10.21873/invivo.13345. PMID: 37905620Free PMC Article
Borrelli de Andreis F, Boškoski I, Mascagni P, Schepis T, Bianchi A, Schinzari G, Annicchiarico BE, Quero G, Tortora G, Alfieri S, Gasbarrini A, Costamagna G, Spada C, Attili F
Pancreatology 2023 Aug;23(5):543-549. Epub 2023 May 22 doi: 10.1016/j.pan.2023.05.004. PMID: 37236853
Crinò SF, Napoleon B, Facciorusso A, Lakhtakia S, Borbath I, Caillol F, Do-Cong Pham K, Rizzatti G, Forti E, Palazzo L, Belle A, Vilmann P, van Laethem JL, Mohamadnejad M, Godat S, Hindryckx P, Benson A, Tacelli M, De Nucci G, Binda C, Kovacevic B, Jacob H, Partelli S, Falconi M, Salvia R, Landoni L; ERASING study collaborators, Larghi A
Clin Gastroenterol Hepatol 2023 Oct;21(11):2834-2843.e2. Epub 2023 Mar 4 doi: 10.1016/j.cgh.2023.02.022. PMID: 36871765
de Carbonnières A, Challine A, Cottereau AS, Coriat R, Soyer P, Abou Ali E, Prat F, Terris B, Bertherat J, Dousset B, Gaujoux S
HPB (Oxford) 2021 Dec;23(12):1799-1806. Epub 2021 Apr 27 doi: 10.1016/j.hpb.2021.04.013. PMID: 33975801
Menegaux F, Schmitt G, Mercadier M, Chigot JP
Am J Surg 1993 Feb;165(2):243-8. doi: 10.1016/s0002-9610(05)80519-7. PMID: 8427405

Diagnosis

Damaskos C, Dimitroulis D, Garmpi A, Antoniou EA, Kouraklis G, Psilopatis I, Mavri M, Diamantis E, Marinos G, Kyriakos G, Farmaki P, Patsouras A, Kontzoglou K, Garmpis N
In Vivo 2023 Nov-Dec;37(6):2402-2408. doi: 10.21873/invivo.13345. PMID: 37905620Free PMC Article
Guerrero-Pérez F, Vilarrasa N, Huánuco LV, Busquets J, Secanella L, Vercher-Conejero JL, Vidal N, Näf Cortés S, Villabona C
Rev Endocr Metab Disord 2023 Dec;24(6):1135-1146. Epub 2023 Jul 12 doi: 10.1007/s11154-023-09824-2. PMID: 37434098
De Angelis C, Pellicano R, Rizzetto M, Repici A
Minerva Gastroenterol Dietol 2011 Jun;57(2):129-37. PMID: 21587152
Vaidakis D, Karoubalis J, Pappa T, Piaditis G, Zografos GN
Hepatobiliary Pancreat Dis Int 2010 Jun;9(3):234-41. PMID: 20525548
Menegaux F, Schmitt G, Mercadier M, Chigot JP
Am J Surg 1993 Feb;165(2):243-8. doi: 10.1016/s0002-9610(05)80519-7. PMID: 8427405

Therapy

Crinò SF, Napoleon B, Facciorusso A, Lakhtakia S, Borbath I, Caillol F, Do-Cong Pham K, Rizzatti G, Forti E, Palazzo L, Belle A, Vilmann P, van Laethem JL, Mohamadnejad M, Godat S, Hindryckx P, Benson A, Tacelli M, De Nucci G, Binda C, Kovacevic B, Jacob H, Partelli S, Falconi M, Salvia R, Landoni L; ERASING study collaborators, Larghi A
Clin Gastroenterol Hepatol 2023 Oct;21(11):2834-2843.e2. Epub 2023 Mar 4 doi: 10.1016/j.cgh.2023.02.022. PMID: 36871765
Marx M, Trosic-Ivanisevic T, Caillol F, Demartines N, Schoepfer A, Pesenti C, Ratone JP, Robert M, Giovannini M, Godat S
Gastrointest Endosc 2022 Jun;95(6):1256-1263. Epub 2021 Dec 10 doi: 10.1016/j.gie.2021.11.045. PMID: 34902374
de Carbonnières A, Challine A, Cottereau AS, Coriat R, Soyer P, Abou Ali E, Prat F, Terris B, Bertherat J, Dousset B, Gaujoux S
HPB (Oxford) 2021 Dec;23(12):1799-1806. Epub 2021 Apr 27 doi: 10.1016/j.hpb.2021.04.013. PMID: 33975801
Mosquera-Klinger G, Carvajal JJ
Rev Esp Enferm Dig 2021 Jan;113(1):48-51. doi: 10.17235/reed.2020.7109/2020. PMID: 33226258
Su AP, Ke NW, Zhang Y, Liu XB, Hu WM, Tian BL, Zhang ZD
J Surg Res 2014 Jan;186(1):126-34. Epub 2013 Aug 19 doi: 10.1016/j.jss.2013.07.051. PMID: 23992857

Prognosis

Damaskos C, Dimitroulis D, Garmpi A, Antoniou EA, Kouraklis G, Psilopatis I, Mavri M, Diamantis E, Marinos G, Kyriakos G, Farmaki P, Patsouras A, Kontzoglou K, Garmpis N
In Vivo 2023 Nov-Dec;37(6):2402-2408. doi: 10.21873/invivo.13345. PMID: 37905620Free PMC Article
Marx M, Trosic-Ivanisevic T, Caillol F, Demartines N, Schoepfer A, Pesenti C, Ratone JP, Robert M, Giovannini M, Godat S
Gastrointest Endosc 2022 Jun;95(6):1256-1263. Epub 2021 Dec 10 doi: 10.1016/j.gie.2021.11.045. PMID: 34902374
Vaidakis D, Karoubalis J, Pappa T, Piaditis G, Zografos GN
Hepatobiliary Pancreat Dis Int 2010 Jun;9(3):234-41. PMID: 20525548
Tseng LJ, Jao YT, Liu SM, Yang TM, Tsai CC, Mo LR
Gastrointest Endosc 2003 Jul;58(1):97-8. doi: 10.1067/mge.2003.287. PMID: 12838231
Menegaux F, Schmitt G, Mercadier M, Chigot JP
Am J Surg 1993 Feb;165(2):243-8. doi: 10.1016/s0002-9610(05)80519-7. PMID: 8427405

Clinical prediction guides

Borrelli de Andreis F, Boškoski I, Mascagni P, Schepis T, Bianchi A, Schinzari G, Annicchiarico BE, Quero G, Tortora G, Alfieri S, Gasbarrini A, Costamagna G, Spada C, Attili F
Pancreatology 2023 Aug;23(5):543-549. Epub 2023 May 22 doi: 10.1016/j.pan.2023.05.004. PMID: 37236853
Crinò SF, Napoleon B, Facciorusso A, Lakhtakia S, Borbath I, Caillol F, Do-Cong Pham K, Rizzatti G, Forti E, Palazzo L, Belle A, Vilmann P, van Laethem JL, Mohamadnejad M, Godat S, Hindryckx P, Benson A, Tacelli M, De Nucci G, Binda C, Kovacevic B, Jacob H, Partelli S, Falconi M, Salvia R, Landoni L; ERASING study collaborators, Larghi A
Clin Gastroenterol Hepatol 2023 Oct;21(11):2834-2843.e2. Epub 2023 Mar 4 doi: 10.1016/j.cgh.2023.02.022. PMID: 36871765
Su AP, Ke NW, Zhang Y, Liu XB, Hu WM, Tian BL, Zhang ZD
J Surg Res 2014 Jan;186(1):126-34. Epub 2013 Aug 19 doi: 10.1016/j.jss.2013.07.051. PMID: 23992857
De Angelis C, Pellicano R, Rizzetto M, Repici A
Minerva Gastroenterol Dietol 2011 Jun;57(2):129-37. PMID: 21587152
Vaidakis D, Karoubalis J, Pappa T, Piaditis G, Zografos GN
Hepatobiliary Pancreat Dis Int 2010 Jun;9(3):234-41. PMID: 20525548

Recent systematic reviews

Guerrero-Pérez F, Vilarrasa N, Huánuco LV, Busquets J, Secanella L, Vercher-Conejero JL, Vidal N, Näf Cortés S, Villabona C
Rev Endocr Metab Disord 2023 Dec;24(6):1135-1146. Epub 2023 Jul 12 doi: 10.1007/s11154-023-09824-2. PMID: 37434098
Su AP, Ke NW, Zhang Y, Liu XB, Hu WM, Tian BL, Zhang ZD
J Surg Res 2014 Jan;186(1):126-34. Epub 2013 Aug 19 doi: 10.1016/j.jss.2013.07.051. PMID: 23992857

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