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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

Synchronous Insulinoma and Glucagonoma: A Review of the Literature.
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
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: 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
Management of Functional Pancreatic Neuroendocrine Neoplasms.
Magi L, Marasco M, Rinzivillo M, Faggiano A, Panzuto F
Curr Treat Options Oncol 2023 Jul;24(7):725-741. Epub 2023 Apr 27 doi: 10.1007/s11864-023-01085-0. PMID: 37103745Free 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
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

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
Nesidioblastosis in adults.
Dravecka I, Lazurova I
Neoplasma 2014;61(3):252-6. doi: 10.4149/neo_2014_047. PMID: 24645840
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

Well-differentiated G1 and G2 pancreatic neuroendocrine tumors: a meta-analysis of published expanded DNA sequencing data.
Andersen KØ, Detlefsen S, Brusgaard K, Christesen HT
Front Endocrinol (Lausanne) 2024;15:1351624. Epub 2024 May 29 doi: 10.3389/fendo.2024.1351624. PMID: 38868744Free PMC Article
Ectopic insulinoma: a systematic review.
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
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

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