Glucagonoma history and symptoms
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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]
- 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, buttocks, perineum, and groin.
- Weight loss
- Glucose intolerance
Less common symptoms include:
Less common symptoms of glucagonoma include:[5][6]
- Cheilosis
- intraabdominal hemorrhage
- Venous thrombosis
- Stomatitis
- Diarrhea
- Polyuria
- Polydipsia
- Polyphagia
- Blurred vision
- Neuropsychiatric manifestations include depression, dementia, psychosis, and agitation
- Dilated cardiomyopathy
- Some patients do not present with hormonal symptoms but present with 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 chromogranins, enolase, pancreatic polypeptide, and ghrelin in patients with metastses.[7]
- Some tumors can develop additional syndromes subsequently due to secretion of more than one hormone.
-
![Close-up view of well demarcated erythematous plaques, with fragile vesicles on gluteal area[8]](/images/6/69/Close-up_view_of_well_demarcated_erythematous_plaques%2C_with_fragile_vesicles_on_gluteal_area..jpg)
Close-up view of well demarcated erythematous plaques, with fragile vesicles on gluteal area[8]
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![Necrolytic migratory erythema with erosion and crust formation[9]](/images/0/08/NEM1.jpg)
Necrolytic migratory erythema with erosion and crust formation[9]
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![Skin eruptionsA) Erythema, scaling, erosions and crusts on the face B) Intense erythema with crusted erosions at perineum C) Polycyclic migratory lesions with scaling advancing borders at groin folds D) Glossitis[10]](/images/4/44/Glucagonoma1.jpg)
Skin eruptionsA) Erythema, scaling, erosions and crusts on the face B) Intense erythema with crusted erosions at perineum C) Polycyclic migratory lesions with scaling advancing borders at groin folds D) Glossitis[10]
![Close-up view of well demarcated erythematous plaques, with fragile vesicles on gluteal area[8]](/index.php/File:Close-up_view_of_well_demarcated_erythematous_plaques,_with_fragile_vesicles_on_gluteal_area..jpg)
![Necrolytic migratory erythema with erosion and crust formation[9]](/index.php/File:NEM1.jpg)
![Skin eruptionsA) Erythema, scaling, erosions and crusts on the face B) Intense erythema with crusted erosions at perineum C) Polycyclic migratory lesions with scaling advancing borders at groin folds D) Glossitis[10]](/index.php/File:Glucagonoma1.jpg)
References
- ↑ 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.
- ↑ Wilkinson DS (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Trans St Johns Hosp Dermatol Soc. 59 (2): 244–50. PMID 4793623.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Wu SL, Bai JG, Xu J, Ma QY, Wu Z (2014). "Necrolytic migratory erythema as the first manifestation of pancreatic neuroendocrine tumor". World J Surg Oncol. 12: 220. doi:10.1186/1477-7819-12-220. PMC 4105234. PMID 25029913.
- ↑ Fang S, Li S, Cai T (2014). "Glucagonoma syndrome: a case report with focus on skin disorders". Onco Targets Ther. 7: 1449–53. doi:10.2147/OTT.S66285. PMC 4140234. PMID 25152626.
- ↑ Erdas E, Aste N, Pilloni L, Nicolosi A, Licheri S, Cappai A; et al. (2012). "Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association?". BMC Cancer. 12: 614. doi:10.1186/1471-2407-12-614. PMC 3543729. PMID 23259638.