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Glucagonoma syndrome

MedGen UID:
4908
Concept ID:
C0017689
Neoplastic Process
Synonyms: Glucagonoma; Weight loss, necrolytic migratory erythema (NME), diabetes, stomatitis, and diarrhea
SNOMED CT: Glucagonoma syndrome (16424000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0030404
Monarch Initiative: MONDO:0019959
Orphanet: ORPHA97280

Definition

An endocrine tumor of the pancreas that secretes excessive amounts of glucagon. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGlucagonoma syndrome
Follow this link to review classifications for Glucagonoma syndrome 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.

Professional guidelines

PubMed

John AM, Schwartz RA
J Eur Acad Dermatol Venereol 2016 Dec;30(12):2016-2022. Epub 2016 Jul 16 doi: 10.1111/jdv.13752. PMID: 27422767
Abreu Velez AM, Howard MS
Dermatol Ther 2010 Nov-Dec;23(6):662-75. doi: 10.1111/j.1529-8019.2010.01371.x. PMID: 21054710
Stacpoole PW
Endocr Rev 1981 Summer;2(3):347-61. doi: 10.1210/edrv-2-3-347. PMID: 6268399

Recent clinical studies

Etiology

de Herder WW, Rehfeld JF, Kidd M, Modlin IM
Best Pract Res Clin Endocrinol Metab 2016 Jan;30(1):3-17. Epub 2015 Oct 23 doi: 10.1016/j.beem.2015.10.004. PMID: 26971840
Singh R, Basturk O, Klimstra DS, Zamboni G, Chetty R, Hussain S, La Rosa S, Yilmaz A, Capelli P, Capella C, Cheng JD, Adsay NV
Am J Surg Pathol 2006 Feb;30(2):194-200. doi: 10.1097/01.pas.0000184819.71752.ad. PMID: 16434893
Warner RR
Gastroenterology 2005 May;128(6):1668-84. doi: 10.1053/j.gastro.2005.03.078. PMID: 15887158
Echenique-Elizondo M, Tuneu Valls A, Elorza Orúe JL, Martinez de Lizarduy I, Ibáñez Aguirre J
JOP 2004 Jul;5(4):179-85. PMID: 15254346
Wermers RA, Fatourechi V, Kvols LK
Mayo Clin Proc 1996 Nov;71(11):1030-8. doi: 10.4065/71.11.1030. PMID: 8917287

Diagnosis

John AM, Schwartz RA
J Eur Acad Dermatol Venereol 2016 Dec;30(12):2016-2022. Epub 2016 Jul 16 doi: 10.1111/jdv.13752. PMID: 27422767
Wewer Albrechtsen NJ, Challis BG, Damjanov I, Holst JJ
Bosn J Basic Med Sci 2016 Feb 1;16(1):1-7. doi: 10.17305/bjbms.2015.794. PMID: 26773171Free PMC Article
Singh R, Basturk O, Klimstra DS, Zamboni G, Chetty R, Hussain S, La Rosa S, Yilmaz A, Capelli P, Capella C, Cheng JD, Adsay NV
Am J Surg Pathol 2006 Feb;30(2):194-200. doi: 10.1097/01.pas.0000184819.71752.ad. PMID: 16434893
Echenique-Elizondo M, Tuneu Valls A, Elorza Orúe JL, Martinez de Lizarduy I, Ibáñez Aguirre J
JOP 2004 Jul;5(4):179-85. PMID: 15254346
Bloom SR, Polak JM
Am J Med 1987 May 29;82(5B):25-36. doi: 10.1016/0002-9343(87)90424-4. PMID: 2884877

Therapy

Yu R
J Clin Endocrinol Metab 2014 Mar;99(3):748-56. Epub 2013 Nov 27 doi: 10.1210/jc.2013-2952. PMID: 24285676
Radny P, Eigentler TK, Soennichsen K, Overkamp D, Raab HR, Viebahn R, Mueller-Horvart C, Sotlar K, Rassner G
J Am Acad Dermatol 2006 Feb;54(2):344-7. doi: 10.1016/j.jaad.2005.05.023. PMID: 16443073
Chastain MA
Am J Med Sci 2001 May;321(5):306-20. doi: 10.1097/00000441-200105000-00003. PMID: 11370794
Arnold R
Eur J Clin Invest 1990 Oct;20 Suppl 1:S82-90. doi: 10.1111/j.1365-2362.1990.tb01782.x. PMID: 1702388
Bloom SR, Polak JM
Am J Med 1987 May 29;82(5B):25-36. doi: 10.1016/0002-9343(87)90424-4. PMID: 2884877

Prognosis

Obi N, Katabami T, Obi R, Odanaka M, Sasano K, Tanaka Y
Endocr J 2009;56(5):715-9. Epub 2009 Apr 11 doi: 10.1507/endocrj.k09e-058. PMID: 19367016
Radny P, Eigentler TK, Soennichsen K, Overkamp D, Raab HR, Viebahn R, Mueller-Horvart C, Sotlar K, Rassner G
J Am Acad Dermatol 2006 Feb;54(2):344-7. doi: 10.1016/j.jaad.2005.05.023. PMID: 16443073
Warner RR
Gastroenterology 2005 May;128(6):1668-84. doi: 10.1053/j.gastro.2005.03.078. PMID: 15887158
Tomassetti P, Migliori M, Lalli S, Campana D, Tomassetti V, Corinaldesi R
Ann Oncol 2001;12 Suppl 2:S95-9. doi: 10.1093/annonc/12.suppl_2.s95. PMID: 11762360
Lewis AE
Int J Dermatol 1979 Jan-Feb;18(1):17-22. doi: 10.1111/j.1365-4362.1979.tb01904.x. PMID: 216645

Clinical prediction guides

Kovács RK, Korom I, Dobozy A, Farkas G, Ormos J, Kemény L
J Cutan Pathol 2006 Mar;33(3):242-5. doi: 10.1111/j.0303-6987.2006.00408.x. PMID: 16466513
Echenique-Elizondo M, Tuneu Valls A, Elorza Orúe JL, Martinez de Lizarduy I, Ibáñez Aguirre J
JOP 2004 Jul;5(4):179-85. PMID: 15254346
Chastain MA
Am J Med Sci 2001 May;321(5):306-20. doi: 10.1097/00000441-200105000-00003. PMID: 11370794
Stacpoole PW, Jaspan J, Kasselberg AG, Halter SA, Polonsky K, Gluck FW, Liljenquist JE, Rabin D
Am J Med 1981 May;70(5):1017-26. doi: 10.1016/0002-9343(81)90858-5. PMID: 6112877
Lewis AE
Int J Dermatol 1979 Jan-Feb;18(1):17-22. doi: 10.1111/j.1365-4362.1979.tb01904.x. PMID: 216645

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