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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

Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance.
Anlauf M, Perren A, Klöppel G
Pathobiology 2007;74(5):279-84. doi: 10.1159/000105810. PMID: 17890894

Recent clinical studies

Etiology

Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Assessment of selective arterial calcium stimulation and hepatic venous sampling to localize insulin-secreting tumours.
Brändle M, Pfammatter T, Spinas GA, Lehmann R, Schmid C
Clin Endocrinol (Oxf) 2001 Sep;55(3):357-62. doi: 10.1046/j.1365-2265.2001.01335.x. PMID: 11589679

Diagnosis

GAD2 Is a Highly Specific Marker for Neuroendocrine Neoplasms of the Pancreas.
Lennartz M, Benjamin Dünnebier N, Höflmayer D, Dwertmann Rico S, Kind S, Reiswich V, Viehweger F, Lutz F, Fraune C, Gorbokon N, Luebke AM, Hube-Magg C, Büscheck F, Menz A, Uhlig R, Krech T, Hinsch A, Burandt E, Sauter G, Simon R, Kluth M, Steurer S, Marx AH, Lebok P, Dum D, Minner S, Jacobsen F, Clauditz TS, Bernreuther C
Am J Surg Pathol 2024 Apr 1;48(4):377-386. Epub 2024 Jan 25 doi: 10.1097/PAS.0000000000002186. PMID: 38271200Free PMC Article
Pancreatic Neuroendocrine Tumor Producing Insulin and Vasopressin.
Alshaikh OM, Yoon JY, Chan BA, Krzyzanowska MK, Butany J, Asa SL, Ezzat S
Endocr Pathol 2018 Mar;29(1):15-20. doi: 10.1007/s12022-017-9492-5. PMID: 28718084
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance.
Anlauf M, Perren A, Klöppel G
Pathobiology 2007;74(5):279-84. doi: 10.1159/000105810. PMID: 17890894
Diagnostic pathology of gastrointestinal and pancreatic neuroendocrine tumours.
Wilander E
Acta Oncol 1989;28(3):363-9. doi: 10.3109/02841868909111207. PMID: 2472824

Therapy

Management of refractory hypoglycaemia in a metastatic neuroendocrine tumour co-secreting serotonin and insulin.
Bhullar JS, Leung JM, Almehthel MS
BMJ Case Rep 2020 Nov 9;13(11) doi: 10.1136/bcr-2020-236659. PMID: 33168531Free PMC Article
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028

Prognosis

Alternative Lengthening of Telomeres and Differential Expression of Endocrine Transcription Factors Distinguish Metastatic and Non-metastatic Insulinomas.
Hackeng WM, Schelhaas W, Morsink FHM, Heidsma CM, van Eeden S, Valk GD, Vriens MR, Heaphy CM, Nieveen van Dijkum EJM, Offerhaus GJA, Dreijerink KMA, Brosens LAA
Endocr Pathol 2020 Jun;31(2):108-118. doi: 10.1007/s12022-020-09611-8. PMID: 32103422Free PMC Article
Non-functioning neuroendocrine pancreatic tumors transforming to malignant insulinomas - four cases and review of the literature.
Juhlin CC, Skoglund S, Juntti-Berggren L, Karlberg M, Calissendorff J
Neuro Endocrinol Lett 2019 Nov;40(4):175-183. PMID: 32087093
Clinical characteristics and management of insulin-producing neuroendocrine carcinomas.
Lowette K, Verslype C, Van Cutsem E
Acta Gastroenterol Belg 2016 Jul-Sep;79(2):321-327. PMID: 27821028
Expression and localization of Reg IV in human neoplastic and non-neoplastic tissues: Reg IV expression is associated with intestinal and neuroendocrine differentiation in gastric adenocarcinoma.
Oue N, Mitani Y, Aung PP, Sakakura C, Takeshima Y, Kaneko M, Noguchi T, Nakayama H, Yasui W
J Pathol 2005 Oct;207(2):185-98. doi: 10.1002/path.1827. PMID: 16086444
Biological aspects of neuroendocrine gastro-enteropancreatic tumours.
Oberg K
Digestion 1996;57 Suppl 1:42-4. doi: 10.1159/000201393. PMID: 8813467

Clinical prediction guides

Alternative Lengthening of Telomeres and Differential Expression of Endocrine Transcription Factors Distinguish Metastatic and Non-metastatic Insulinomas.
Hackeng WM, Schelhaas W, Morsink FHM, Heidsma CM, van Eeden S, Valk GD, Vriens MR, Heaphy CM, Nieveen van Dijkum EJM, Offerhaus GJA, Dreijerink KMA, Brosens LAA
Endocr Pathol 2020 Jun;31(2):108-118. doi: 10.1007/s12022-020-09611-8. PMID: 32103422Free PMC Article
Serine protease inhibitor 8 is a novel immunohistochemical marker for neuroendocrine tumors of the pancreas.
de Koning PJ, Bovenschen N, Broekhuizen R, Lips CJ, Kummer JA
Pancreas 2009 May;38(4):461-7. doi: 10.1097/MPA.0b013e318194fcc2. PMID: 19188865
Endocrine precursor lesions and microadenomas of the duodenum and pancreas with and without MEN1: criteria, molecular concepts and clinical significance.
Anlauf M, Perren A, Klöppel G
Pathobiology 2007;74(5):279-84. doi: 10.1159/000105810. PMID: 17890894
Diagnostic pathology of gastrointestinal and pancreatic neuroendocrine tumours.
Wilander E
Acta Oncol 1989;28(3):363-9. doi: 10.3109/02841868909111207. PMID: 2472824

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