Glucagonoma history and symptoms: Difference between revisions

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{{Glucagonoma}}
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==Overview==
==Overview==
Symptoms of glucagonoma include [[necrolytic migratory erythema]], [[weight loss]], [[glucose intolerance]], [[cheilosis]], [[stomatitis]], [[diarrhea]], [[polyuria]], and [[polydipsia]]. A positive family history of [[multiple endocrine neoplasia type 1]] may be present.


==History and Symptoms==
==History==
* [[Necrolytic migratory erythema]] (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower [[abdomen]], [[buttock]]s, [[perineum]], and [[groin]].
* When evaluating a patient for glucagonoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough review of past medical history.
* Other specific areas of focus when obtaining the history include the family history of predisposing [[genetic disorders]] such as [[multiple endocrine neoplasia type 1|multiple endocrine neoplasia type 1.]]
 
==Symptoms==
 
===Common symptoms of glucagonoma===
Common symptoms of glucagonoma include:<ref name="pmid8606627">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627  }} </ref><ref name="pmid4793623">{{cite journal| author=Wilkinson DS| title=Necrolytic migratory erythema with carcinoma of the pancreas. | journal=Trans St Johns Hosp Dermatol Soc | year= 1973 | volume= 59 | issue= 2 | pages= 244-50 | pmid=4793623 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=4793623  }}</ref><ref name="pmid86066272">{{cite journal| author=Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV| title=The glucagonoma syndrome. Clinical and pathologic features in 21 patients. | journal=Medicine (Baltimore) | year= 1996 | volume= 75 | issue= 2 | pages= 53-63 | pmid=8606627 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8606627  }}</ref><ref name="pmid6268399">{{cite journal| author=Stacpoole PW| title=The glucagonoma syndrome: clinical features, diagnosis, and treatment. | journal=Endocr Rev | year= 1981 | volume= 2 | issue= 3 | pages= 347-61 | pmid=6268399 | doi=10.1210/edrv-2-3-347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6268399  }}</ref>
*[[Necrolytic migratory erythema]] (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of [[Erythematous rash|erythematous blisters]] and swelling across areas subject to greater friction and pressure, including the lower [[abdomen]], [[buttock]]s, [[perineum]], and [[groin]].
*[[Weight loss]]
*[[Glucose intolerance|Glucose intolerance]]
 
=== Less common symptoms include: ===
Less common symptoms of glucagonoma include:<ref name="pmid62683992">{{cite journal| author=Stacpoole PW| title=The glucagonoma syndrome: clinical features, diagnosis, and treatment. | journal=Endocr Rev | year= 1981 | volume= 2 | issue= 3 | pages= 347-61 | pmid=6268399 | doi=10.1210/edrv-2-3-347 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6268399  }}</ref><ref name="pmid15201270">{{cite journal| author=Chang-Chretien K, Chew JT, Judge DP| title=Reversible dilated cardiomyopathy associated with glucagonoma. | journal=Heart | year= 2004 | volume= 90 | issue= 7 | pages= e44 | pmid=15201270 | doi=10.1136/hrt.2004.036905 | pmc=1768315 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15201270  }}</ref><ref name="pmid22350605">{{cite journal| author=Cheema A, Weber J, Strosberg JR| title=Incidental detection of pancreatic neuroendocrine tumors: an analysis of incidence and outcomes. | journal=Ann Surg Oncol | year= 2012 | volume= 19 | issue= 9 | pages= 2932-6 | pmid=22350605 | doi=10.1245/s10434-012-2285-7 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22350605  }}</ref>
*[[Cheilosis]]
*[[Hemorrhage retroperitoneal|Intraabdominal hemorrhage]]
*[[Venous thrombosis]]
*[[Stomatitis]]
*[[Diarrhea]]
*[[Polyuria]]
*[[Polydipsia]]
*[[Polyphagia]]
*[[Blurred vision]]
*[[Neuropsychiatric|Neuropsychiatric manifestations]] include [[depression]], [[dementia]], [[psychosis]], and [[agitation]]
*[[Dilated cardiomyopathy]]
 
* Some patients do not present with [[Hormonal|hormonal symptoms]] but present with [[Metastasis|metastatic symptoms]] in other [[organs]] and mainly the [[liver]].
**These symptoms include [[jaundice]], [[edema]], and [[abdominal pain]].
**There is usually high level of substances such as [[Chromogranin|chromogranins]], [[enolase]], [[pancreatic polypeptide]], and [[ghrelin]] in patients with [[Metastasis|metastases]].
 
* Some [[tumors]] can develop additional syndromes subsequently due to secretion of more than one [[hormone]].


==References==
==References==
{{reflist|2}}
{{reflist|2}}
 
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Latest revision as of 22:31, 30 May 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2], Mohammed Abdelwahed M.D[3]

Overview

Symptoms of glucagonoma include necrolytic migratory erythema, weight loss, glucose intolerance, cheilosis, stomatitis, diarrhea, polyuria, and polydipsia. A positive family history of multiple endocrine neoplasia type 1 may be present.

History

  • When evaluating a patient for glucagonoma, you should take a detailed history of the presenting symptom (onset, duration, and progression), other associated symptoms, and a thorough review of past medical history.
  • Other specific areas of focus when obtaining the history include the family history of predisposing genetic disorders such as multiple endocrine neoplasia type 1.

Symptoms

Common symptoms of glucagonoma

Common symptoms of glucagonoma include:[1][2][3][4]

Less common symptoms include:

Less common symptoms of glucagonoma include:[5][6][7]

  • Some tumors can develop additional syndromes subsequently due to secretion of more than one hormone.

References

  1. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV (1996). "The glucagonoma syndrome. Clinical and pathologic features in 21 patients". Medicine (Baltimore). 75 (2): 53–63. PMID 8606627.
  2. Wilkinson DS (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Trans St Johns Hosp Dermatol Soc. 59 (2): 244–50. PMID 4793623.
  3. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV (1996). "The glucagonoma syndrome. Clinical and pathologic features in 21 patients". Medicine (Baltimore). 75 (2): 53–63. PMID 8606627.
  4. Stacpoole PW (1981). "The glucagonoma syndrome: clinical features, diagnosis, and treatment". Endocr Rev. 2 (3): 347–61. doi:10.1210/edrv-2-3-347. PMID 6268399.
  5. Stacpoole PW (1981). "The glucagonoma syndrome: clinical features, diagnosis, and treatment". Endocr Rev. 2 (3): 347–61. doi:10.1210/edrv-2-3-347. PMID 6268399.
  6. Chang-Chretien K, Chew JT, Judge DP (2004). "Reversible dilated cardiomyopathy associated with glucagonoma". Heart. 90 (7): e44. doi:10.1136/hrt.2004.036905. PMC 1768315. PMID 15201270.
  7. Cheema A, Weber J, Strosberg JR (2012). "Incidental detection of pancreatic neuroendocrine tumors: an analysis of incidence and outcomes". Ann Surg Oncol. 19 (9): 2932–6. doi:10.1245/s10434-012-2285-7. PMID 22350605.

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