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Pancreatic insulin-producing neuroendocrine tumor

MedGen UID:
1844884
•Concept ID:
C5848150
•
Neoplastic Process
Synonyms: beta cell neoplasm; Beta Cell Tumor; Beta cell tumor; Beta Cell Tumor of Pancreas; Beta cell tumor of pancreas; Beta Cell Tumor of the Pancreas; Beta cell tumor of the pancreas; beta cell tumor of the pancreas; Beta cell tumour; Beta cell tumour of pancreas; beta cell tumour of the pancreas; Beta cell tumour of the pancreas; Insulin-Producing Islet Cell Tumor; insulin-producing islet cell tumor; insulin-producing islet cell tumour; Insulin-Producing Tumor of Islet Cells; insulin-producing tumor of islet cells; Insulin-Producing Tumor of the Islet Cells; insulin-producing tumor of the islet cells; insulin-producing tumour of islet cells; insulin-producing tumour of the islet cells; Insulinoma; Pancreatic Beta Cell Tumor; pancreatic Beta cell tumor; pancreatic Beta cell tumour; Pancreatic Insulin Producing Neoplasm; pancreatic insulin producing neoplasm; Pancreatic Insulin Producing NET; pancreatic insulin producing NET; Pancreatic Insulin Producing Tumor; pancreatic insulin producing tumor; pancreatic insulin producing tumour; Pancreatic Insulin-Producing Neuroendocrine Tumor; pancreatic insulin-producing neuroendocrine tumor; pancreatic insulin-producing tumor; pancreatic insulin-producing tumour
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0005048
Orphanet: ORPHA97279

Definition

A form of functioning pancreatic neuroendocrine tumor characterized most commonly by a solitary, small pancreatic lesion that causes hyperinsulinemic hypoglycemia. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic insulin-producing neuroendocrine tumor

Professional guidelines

PubMed

Insulinomas: from diagnosis to treatment. A review of the literature.
Giannis D, Moris D, Karachaliou GS, Tsilimigras DI, Karaolanis G, Papalampros A, Felekouras E
J BUON 2020 May-Jun;25(3):1302-1314. PMID: 32862570
Diagnosis and management of insulinoma.
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Recent clinical studies

Etiology

Insulinomatosis: new aspects.
Christ E, Iacovazzo D, Korbonits M, Perren A
Endocr Relat Cancer 2023 Jun 1;30(6) Epub 2023 May 16 doi: 10.1530/ERC-22-0327. PMID: 36952647
Endoscopic Ultrasound-guided Radiofrequency Ablation Versus Surgical Resection for Treatment of Pancreatic Insulinoma.
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
Rare causes of hypoglycemia in adults.
Douillard C, Jannin A, Vantyghem MC
Ann Endocrinol (Paris) 2020 Jun;81(2-3):110-117. Epub 2020 Apr 10 doi: 10.1016/j.ando.2020.04.003. PMID: 32409005
Pancreatic neuroendocrine tumors: contemporary diagnosis and management.
Parbhu SK, Adler DG
Hosp Pract (1995) 2016 Aug;44(3):109-19. Epub 2016 Jul 18 doi: 10.1080/21548331.2016.1210474. PMID: 27404266
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Diagnosis

Approach to the Patient: Insulinoma.
Hofland J, Refardt JC, Feelders RA, Christ E, de Herder WW
J Clin Endocrinol Metab 2024 Mar 15;109(4):1109-1118. doi: 10.1210/clinem/dgad641. PMID: 37925662Free PMC Article
Clinical Presentation and Diagnostic Approach to Hypoglycemia in Adults Without Diabetes Mellitus.
Palani G, Stortz E, Moheet A
Endocr Pract 2023 Apr;29(4):286-294. Epub 2022 Dec 2 doi: 10.1016/j.eprac.2022.11.010. PMID: 36464132
Insulinomas: from diagnosis to treatment. A review of the literature.
Giannis D, Moris D, Karachaliou GS, Tsilimigras DI, Karaolanis G, Papalampros A, Felekouras E
J BUON 2020 May-Jun;25(3):1302-1314. PMID: 32862570
Diagnosis and management of insulinoma.
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Insulinoma.
Mathur A, Gorden P, Libutti SK
Surg Clin North Am 2009 Oct;89(5):1105-21. doi: 10.1016/j.suc.2009.06.009. PMID: 19836487Free PMC Article

Therapy

Approach to the Patient: Insulinoma.
Hofland J, Refardt JC, Feelders RA, Christ E, de Herder WW
J Clin Endocrinol Metab 2024 Mar 15;109(4):1109-1118. doi: 10.1210/clinem/dgad641. PMID: 37925662Free PMC Article
Endoscopic Ultrasound-guided Radiofrequency Ablation Versus Surgical Resection for Treatment of Pancreatic Insulinoma.
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
Exendin-4 analogs in insulinoma theranostics.
Jansen TJP, van Lith SAM, Boss M, Brom M, Joosten L, Béhé M, Buitinga M, Gotthardt M
J Labelled Comp Radiopharm 2019 Aug;62(10):656-672. doi: 10.1002/jlcr.3750. PMID: 31070270Free PMC Article
Pancreatic neuroendocrine tumors.
Krampitz GW, Norton JA
Curr Probl Surg 2013 Nov;50(11):509-45. doi: 10.1067/j.cpsurg.2013.08.001. PMID: 24206780
Pancreatic neuroendocrine tumors: clinical features, diagnosis and medical treatment: advances.
Ito T, Igarashi H, Jensen RT
Best Pract Res Clin Gastroenterol 2012 Dec;26(6):737-53. doi: 10.1016/j.bpg.2012.12.003. PMID: 23582916Free PMC Article

Prognosis

Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors.
Anderson CW, Bennett JJ
Surg Oncol Clin N Am 2016 Apr;25(2):363-74. Epub 2016 Feb 18 doi: 10.1016/j.soc.2015.12.003. PMID: 27013370
Imaging of pancreatic neoplasms.
Balachandran A, Bhosale PR, Charnsangavej C, Tamm EP
Surg Oncol Clin N Am 2014 Oct;23(4):751-88. doi: 10.1016/j.soc.2014.07.002. PMID: 25246049
Pancreatic neuroendocrine tumors.
Krampitz GW, Norton JA
Curr Probl Surg 2013 Nov;50(11):509-45. doi: 10.1067/j.cpsurg.2013.08.001. PMID: 24206780
Diagnosis and management of insulinoma.
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Insulinoma.
Grant CS
Surg Oncol Clin N Am 1998 Oct;7(4):819-44. PMID: 9735136

Clinical prediction guides

Endoscopic Ultrasound-guided Radiofrequency Ablation Versus Surgical Resection for Treatment of Pancreatic Insulinoma.
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
Sporadic pancreatic neuroendocrine tumor: Surgery of the primary tumor.
Deguelte S, de Mestier L, Hentic O, Cros J, Lebtahi R, Hammel P, Kianmanesh R
J Visc Surg 2018 Dec;155(6):483-492. Epub 2018 Nov 15 doi: 10.1016/j.jviscsurg.2018.08.010. PMID: 30448206
Imaging of pancreatic neoplasms.
Balachandran A, Bhosale PR, Charnsangavej C, Tamm EP
Surg Oncol Clin N Am 2014 Oct;23(4):751-88. doi: 10.1016/j.soc.2014.07.002. PMID: 25246049
Diagnosis and management of insulinoma.
Okabayashi T, Shima Y, Sumiyoshi T, Kozuki A, Ito S, Ogawa Y, Kobayashi M, Hanazaki K
World J Gastroenterol 2013 Feb 14;19(6):829-37. doi: 10.3748/wjg.v19.i6.829. PMID: 23430217Free PMC Article
Hypoglycemia in adults.
Virally ML, Guillausseau PJ
Diabetes Metab 1999 Dec;25(6):477-90. PMID: 10633872

Recent systematic reviews

Efficacy and Safety of Endoscopic Ultrasound-Guided Radiofrequency Ablation for Pancreatic Neuroendocrine Tumors: A Systematic Review and Metanalysis.
Armellini E, Facciorusso A, Crinò SF
Medicina (Kaunas) 2023 Feb 14;59(2) doi: 10.3390/medicina59020359. PMID: 36837560Free PMC Article
Proteomics of Pancreatic Neuroendocrine Tumors: A Systematic Review.
Maiorano BA, Schinzari G, Chiloiro S, Visconti F, Milardi D, Bianchi A
Protein Pept Lett 2020;27(12):1276-1287. doi: 10.2174/0929866527666200504122116. PMID: 31804164
Cystic pancreatic neuroendocrine tumors (cPNETs): a systematic review and meta-analysis of case series.
Hurtado-Pardo L, Cienfuegos JA, Ruiz-Canela M, Panadero P, Benito A, Hernández Lizoain JL
Rev Esp Enferm Dig 2017 Nov;109(11):778-787. doi: 10.17235/reed.2017.5044/2017. PMID: 29072081
A Systematic Review of Proinsulin-Secreting Pancreatic Neuroendocrine Tumors.
Murtha TD, Lupsa BC, Majumdar S, Jain D, Salem RR
J Gastrointest Surg 2017 Aug;21(8):1335-1341. Epub 2017 May 16 doi: 10.1007/s11605-017-3428-8. PMID: 28510792
A systematic review of localization, surgical treatment options, and outcome of insulinoma.
Mehrabi A, Fischer L, Hafezi M, Dirlewanger A, Grenacher L, Diener MK, Fonouni H, Golriz M, Garoussi C, Fard N, Rahbari NN, Werner J, Büchler MW
Pancreas 2014 Jul;43(5):675-86. doi: 10.1097/MPA.0000000000000110. PMID: 24921202

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