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   MeshID        = D005935 |
   MeshID        = D005935 |
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{{Glucagonoma}}
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{{EH}}
==[[Glucagonoma overview|Overview]]==


==Overview==
==[[Glucagonoma historical perspective|Historical Perspective]]==
A '''glucagonoma''' is a rare [[tumor]] of the [[alpha cell]]s of the [[pancreas]] that results in up to a 1000-fold overproduction of the [[hormone]] [[glucagon]]. Alpha cell tumors are commonly associated with glucagonoma syndrome, though similar symptoms are present in cases of pseudoglucagonoma syndrome in the absence of a glucagon-secreting tumor.


== History ==
==[[Glucagonoma pathophysiology|Pathophysiology]]==
Fewer than 250 cases of glucagonoma have been described in the literature since their first description by Becker in 1942. Because of its rarity (fewer than one in 20 million worldwide), long-term survival rates are as yet unknown.


== Symptoms ==
==[[Glucagonoma epidemiology and demographics|Epidemiology & Demographics]]==
The primary [[physiology|physiological]] effect of glucagonoma is an overproduction of the [[peptide]] hormone [[glucagon]], which enhances [[blood glucose]] levels through the activation of [[catabolism|catabolic]] processes including [[gluconeogenesis]] and [[lipolysis]]. Gluconeogenesis produces [[glucose]] from [[protein]] and [[amino acid]] materials; lipolysis is the breakdown of [[adipose tissue|fat]]. The net result is hyperglucagonemia, decreased blood levels of [[amino acid]]s (hypoaminoacidemia), [[anemia]], [[diarrhea]], and weight loss of 5-15 [[kilogram|kg]].


[[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]].
==[[Glucagonoma risk factors|Risk Factors]]==


[[Diabetes mellitus]] also frequently results from the [[insulin]] and [[glucagon]] imbalance that occurs in glucagonoma.<ref>{{cite journal |author=Koike N, Hatori T, Imaizumi T, ''et al'' |title=Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus |journal=Journal of hepato-biliary-pancreatic surgery |volume=10 |issue=1 |pages=101-5 |year=2003 |pmid=12918465 |doi=}}</ref> Diabetes mellitus is present in 80-90% of cases of glucagonoma, and is exacerbated by preexisting [[insulin resistance]].
==[[Glucagonoma screening|Screening]]==


== Diagnosis ==
==[[Glucagonoma causes|Causes of Glucagonoma]]==
A [[blood serum]] glucagon concentration of 1000 pg/mL or greater is indicative of glucagonoma (the normal range is 50-200 pg/mL).


Blood tests may also reveal abnormally low concentrations of amino acids, [[zinc]], and [[essential fatty acid]]s, which are thought to play a role in the development of NME. Skin [[biopsy|biopsies]] may also be taken to confirm the presence of NME.
==[[Glucagonoma differential diagnosis|Differentiating Glucagonoma from other Diseases]]==


A [[complete blood count|CBC]] can uncover anemia, which is an abnormally low level of [[hemoglobin]].
==[[Glucagonoma natural history|Natural History, Complications & Prognosis]]==


The tumor itself may be localized by any number of radiographic modalities, including [[angiography]], [[computed tomography|CT]], [[magnetic resonance imaging|MRI]], [[positron emission tomography|PET]], and [[endoscopic ultrasound]]. [[Laparotomy]] is useful for obtaining [[histology|histologic]] samples for analysis and confirmation of the glucagonoma.
==Diagnosis==
[[Glucagonoma history and symptoms|History & Symptoms]] | [[Glucagonoma physical examination|Physical Examination]] | [[Glucagonoma laboratory tests|Lab Tests]] | [[Glucagonoma electrocardiogram|Electrocardiogram]] | [[Glucagonoma chest x ray|Chest X Ray]] | [[Glucagonoma CT|CT]] | [[Glucagonoma MRI|MRI]] | [[Glucagonoma echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Glucagonoma other imaging findings|Other Imaging Findings]] | [[Glucagonoma other diagnostic studies|Other Diagnostic Studies]]


== Treatment ==
==Treatment==
Heightened glucagon secretion can be treated with the administration of [[Somatostatin|octreotide, a somatostatin analog]], which inhibits the release of glucagon.<ref>{{cite journal |author=Moattari AR, Cho K, Vinik AI |title=Somatostatin analogue in treatment of coexisting glucagonoma and pancreatic pseudocyst: dissociation of responses |journal=Surgery |volume=108 |issue=3 |pages=581-7 |year=1990 |pmid=2168587 |doi=}}</ref> [[Doxorubicin]] and [[streptozotocin]] have also been used successfully to selectively damage alpha cells of the pancreatic islets. These do not destroy the tumor, but help to minimize progression of symptoms.
[[Glucagonoma medical therapy|Medical Therapy]] | [[Glucagonoma surgery|Surgery]] | [[Glucagonoma primary prevention|Primary Prevention]] | [[Glucagonoma secondary prevention|Secondary Prevention]] | [[Glucagonoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Glucagonoma future or investigational therapies|Future or Investigational Therapies]]
 
The only curative therapy for glucagonoma is [[surgery|surgical]] resection, where the tumor is removed. Resection has been known to reverse symptoms in some patients.


== References ==
== References ==
{{reflist|2}}
{{reflist|2}}
{{Epithelial neoplasms}}
{{Epithelial neoplasms}}
[[Category:Types of cancer]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Oncology]]
[[Category:Mature chapter]]
{{SIB}}
[[de:Glucagonom]]


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Revision as of 20:23, 18 January 2012

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Glucagonoma
ICD-10 C25.4
ICD-9 157.4, 211.7
ICD-O: 8152
DiseasesDB 5257
MeSH D005935

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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Causes of Glucagonoma

Differentiating Glucagonoma from other Diseases

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