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Islet Cell Adenoma

MedGen UID:
9562
•Concept ID:
C0022134
•
Neoplastic Process
Synonyms: Adenoma, Islet Cell; Adenomas, Islet Cell; Islet Cell Adenomas
SNOMED CT: Islet cell adenoma (76345009); Islet cell tumor, benign (76345009); Pancreatic endocrine tumor, benign (76345009)

Definition

A benign endocrine neoplasm arising from the pancreas. It is separated from the normal pancreatic tissues by a thin collagenous capsule. It may secrete a hormone (e.g. insulin, gastrin) or it may be non-functional. [from NCI]

Professional guidelines

PubMed

Surgical treatment of organic pancreatic hypoglycemia in the pediatric age.
Carcassonne M, DeLarue A, LeTourneau JN
J Pediatr Surg 1983 Feb;18(1):75-9. doi: 10.1016/s0022-3468(83)80277-2. PMID: 6300364
Surgical management of islet-cell adenoma in infancy.
Rich RH, Dehner LP, Okinaga K, Deeb LC, Ulstrom RA, Leonard AS
Surgery 1978 Oct;84(4):519-26. PMID: 211657
Medical treatment of endogenous organic hyperinsulinism.
Scandellari C, Zaccaria M, Sicolo N, Casara D, Erle G, Federspil G
Horm Metab Res 1976;Suppl 6:46-54. PMID: 179929

Recent clinical studies

Etiology

Pancreatic islet cell tumors in adolescents and young adults.
Waters AM, Maizlin II, Russell RT, Dellinger M, Gow KW, Goldin A, Goldfarb M, Nuchtern JG, Langer M, Vasudevan SA, Doski JJ, Raval M, Beierle EA
J Pediatr Surg 2019 Oct;54(10):2103-2106. Epub 2019 Mar 27 doi: 10.1016/j.jpedsurg.2019.01.060. PMID: 30954230
Pancreatic neuroendocrine tumours.
O'Grady HL, Conlon KC
Eur J Surg Oncol 2008 Mar;34(3):324-32. Epub 2007 Oct 29 doi: 10.1016/j.ejso.2007.07.209. PMID: 17967523
Calcitonin-secreting VIPoma.
Jackson C, Buchman AL
Dig Dis Sci 2005 Dec;50(12):2203-6. doi: 10.1007/s10620-005-3034-6. PMID: 16416161
Islet cell tumors of the pancreas.
Bieligk S, Jaffe BM
Surg Clin North Am 1995 Oct;75(5):1025-40. doi: 10.1016/s0039-6109(16)46744-7. PMID: 7660247
Total pancreatectomy.
ReMine WH, Priestley JT, Judd ES, King JN
Ann Surg 1970 Oct;172(4):595-604. doi: 10.1097/00000658-197010000-00006. PMID: 4318798Free PMC Article

Diagnosis

Pancreatic neuroendocrine tumours.
O'Grady HL, Conlon KC
Eur J Surg Oncol 2008 Mar;34(3):324-32. Epub 2007 Oct 29 doi: 10.1016/j.ejso.2007.07.209. PMID: 17967523
Somatostatinomas, PPomas, neurotensinomas.
Vinik AI, Strodel WE, Eckhauser FE, Moattari AR, Lloyd R
Semin Oncol 1987 Sep;14(3):263-81. PMID: 2820062
Misdiagnosis of insulinoma.
Best JD, Chisholm DJ, Alford FP
Med J Aust 1978 Jul 1;2(1):10-2. doi: 10.5694/j.1326-5377.1978.tb131303.x. PMID: 210364
Insulinomas.
Nardi GL
Adv Surg 1971;5:103-8. PMID: 4330848
LEUCINE-INDUCED HYPOGLYCEMIA.
FAJANS SS
N Engl J Med 1965 Jun 10;272:1224-7. doi: 10.1056/NEJM196506102722308. PMID: 14284995

Therapy

Islet Cell Tumors of the Pancreas.
Amin S, Kim MK
Gastroenterol Clin North Am 2016 Mar;45(1):83-100. Epub 2016 Jan 13 doi: 10.1016/j.gtc.2015.10.007. PMID: 26895682
Glucagonoma syndrome.
Bloom SR, Polak JM
Am J Med 1987 May 29;82(5B):25-36. doi: 10.1016/0002-9343(87)90424-4. PMID: 2884877
Pancreatic cholera.
Gardner JD
West J Med 1975 Oct;123(4):309-10. PMID: 173092Free PMC Article
Diazoxide.
Br Med J 1972 Nov 18;4(5837):417-8. PMID: 4344353Free PMC Article
Glucagon.
Br Med J 1971 Aug 26;3(5773):524-5. PMID: 4327680Free PMC Article

Prognosis

Gastrointestinal hormones stimulate growth of Foregut Neuroendocrine Tumors by transactivating the EGF receptor.
Di Florio A, Sancho V, Moreno P, Delle Fave G, Jensen RT
Biochim Biophys Acta 2013 Mar;1833(3):573-82. Epub 2012 Dec 4 doi: 10.1016/j.bbamcr.2012.11.021. PMID: 23220008Free PMC Article
Islet cell tumours.
Ekeblad S
Adv Exp Med Biol 2010;654:771-89. doi: 10.1007/978-90-481-3271-3_34. PMID: 20217524
Pancreatic neuroendocrine tumours.
O'Grady HL, Conlon KC
Eur J Surg Oncol 2008 Mar;34(3):324-32. Epub 2007 Oct 29 doi: 10.1016/j.ejso.2007.07.209. PMID: 17967523
The glucagonoma syndrome.
Lewis AE
Int J Dermatol 1979 Jan-Feb;18(1):17-22. doi: 10.1111/j.1365-4362.1979.tb01904.x. PMID: 216645
Total pancreatectomy.
ReMine WH, Priestley JT, Judd ES, King JN
Ann Surg 1970 Oct;172(4):595-604. doi: 10.1097/00000658-197010000-00006. PMID: 4318798Free PMC Article

Clinical prediction guides

Islet Tumors: Resect or Follow?
Sinclair TJ, Poultsides GA
Adv Surg 2020 Sep;54:69-88. Epub 2020 Jun 17 doi: 10.1016/j.yasu.2020.04.004. PMID: 32713440
Update on pancreatic endocrine tumors.
Frankel WL
Arch Pathol Lab Med 2006 Jul;130(7):963-6. doi: 10.5858/2006-130-963-UOPET. PMID: 16831051
Oncogenic co-operation in beta-cell tumorigenesis.
Pelengaris S, Khan M
Endocr Relat Cancer 2001 Dec;8(4):307-14. doi: 10.1677/erc.0.0080307. PMID: 11733227
VIPoma syndrome.
Krejs GJ
Am J Med 1987 May 29;82(5B):37-48. doi: 10.1016/0002-9343(87)90425-6. PMID: 3035922
The glucagonoma syndrome.
Lewis AE
Int J Dermatol 1979 Jan-Feb;18(1):17-22. doi: 10.1111/j.1365-4362.1979.tb01904.x. PMID: 216645

Recent systematic reviews

Postoperative Complications, In-Hospital Mortality and 5-Year Survival After Surgical Resection for Patients with a Pancreatic Neuroendocrine Tumor: A Systematic Review.
Jilesen AP, van Eijck CH, in't Hof KH, van Dieren S, Gouma DJ, van Dijkum EJ
World J Surg 2016 Mar;40(3):729-48. doi: 10.1007/s00268-015-3328-6. PMID: 26661846Free PMC Article

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