Glucagonoma pathophysiology: Difference between revisions

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*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 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]].
*[[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]] 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>
*Pathogenesis of [[necrolytic migratory erythema]] is ill defined. Postulated mechanism for necrolytic migratory erythema involves combined effect of hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, hypoaminoacidemia, and liver disease, that leads to excessive inflammation in the epidermis in response to trauma and to the necrolysis observed in necrolytic migratory erythema.<ref>Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.</ref><ref name="pmid9591806">{{cite journal| author=Mullans EA, Cohen PR| title=Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. | journal=J Am Acad Dermatol | year= 1998 | volume= 38 | issue= 5 Pt 2 | pages= 866-73 | pmid=9591806 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9591806  }} </ref>
*Pathogenesis of [[necrolytic migratory erythema]] is ill defined. The postulated mechanism for necrolytic migratory erythema involves the combined effect of hyperglucagonemia, [[zinc deficiency]], [[fatty acid]] deficiency, hypoaminoacidemia, and liver disease, that lead to excessive inflammation in the epidermis in response to trauma and to the necrolysis.<ref>Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.</ref><ref name="pmid9591806">{{cite journal| author=Mullans EA, Cohen PR| title=Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema. | journal=J Am Acad Dermatol | year= 1998 | volume= 38 | issue= 5 Pt 2 | pages= 866-73 | pmid=9591806 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9591806  }} </ref>


==Microscopic Pathology==
==Microscopic Pathology==

Revision as of 15:24, 16 October 2015

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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]

Overview

A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in the overproduction of the hormone glucagon. On microscopic histopathological analysis, findings of glucagonoma are epidermal necrosis, subcorneal pustules, either isolated or associated with necrosis of the epidermis, confluent parakeratosis, epidermal hyperplasia and marked papillary dermal angioplasia, and suppurative folliculitis.

Pathophysiology

  • A glucagonoma is a rare tumor of the alpha cells of the pancreas that results in the 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.[1]
  • The primary physiological effect of glucagonoma is an overproduction of the peptide hormone glucagon, which enhances blood glucose levels through the activation of catabolic processes including gluconeogenesis and lipolysis. Gluconeogenesis produces glucose from protein and amino acid materials, lipolysis is the breakdown of fat.
  • Diabetes mellitus also frequently results from the insulin and glucagon imbalance that occurs in glucagonoma.[2]
  • Pathogenesis of necrolytic migratory erythema is ill defined. The postulated mechanism for necrolytic migratory erythema involves the combined effect of hyperglucagonemia, zinc deficiency, fatty acid deficiency, hypoaminoacidemia, and liver disease, that lead to excessive inflammation in the epidermis in response to trauma and to the necrolysis.[3][4]

Microscopic Pathology

On microscopic histopathological analysis, findings of glucagonoma are:[5]

  • Epidermal necrosis
  • Subcorneal pustules, either isolated or associated with necrosis of the epidermis
  • Confluent parakeratosis
  • Epidermal hyperplasia, and marked papillary dermal angioplasia
  • Suppurative folliculitis

Images

  • Confluent epidermal necrosis (high mag)[6]

    Confluent epidermal necrosis (high mag)[6]

  • Confluent epidermal necrosis (very high mag)[6]

    Confluent epidermal necrosis (very high mag)[6]

  • Confluent epidermal necrosis (intermed mag)[6]

    Confluent epidermal necrosis (intermed mag)[6]

  • Confluent epidermal necrosis (low mag)[6]

    Confluent epidermal necrosis (low mag)[6]

  • (A) Skin lesions affecting pretibial area. (B) Skin biopsy in necrolytic migratory erythema showing a zone of necrolysis and vacuolated keratinocytes[7]

    (A) Skin lesions affecting pretibial area. (B) Skin biopsy in necrolytic migratory erythema showing a zone of necrolysis and vacuolated keratinocytes[7]

  • Skin biopsy in necrolytic migratory erythema showing a large zone of necrolysis in the upper epidermis (arrow)[8]

    Skin biopsy in necrolytic migratory erythema showing a large zone of necrolysis in the upper epidermis (arrow)[8]

  • A) Psoriasiform hyperplasia of the epidermis with overlying parakeratosis and mild perivascular infiltrate of lymphocytes in the upper dermis (HE 5 X). B) Vascular dilatation (HE 20 X).[9]

    A) Psoriasiform hyperplasia of the epidermis with overlying parakeratosis and mild perivascular infiltrate of lymphocytes in the upper dermis (HE 5 X). B) Vascular dilatation (HE 20 X).[9]

  • Specimen from distal splenopancreatectomy.A) The neoplasia is located in the inferior border of the pancreas (arrow); it shows an exophytic growth but appears well circumscribed. B) The cut surface is whitish-yellow in color with focal areas of hemorrhage.[9]

    Specimen from distal splenopancreatectomy.A) The neoplasia is located in the inferior border of the pancreas (arrow); it shows an exophytic growth but appears well circumscribed. B) The cut surface is whitish-yellow in color with focal areas of hemorrhage.[9]

  • Histopathological examination of the pancreatic tumor.A) The tumor appears encapsulated and composed of polygonal cells with trabecular or ribbon-like proliferation (HE 5 X). B) At immunohistochemistry, neoplastic cells showed an intense diffuse staining for glucagon (Anti-glucagon antibody 5 X)[9]

    Histopathological examination of the pancreatic tumor.A) The tumor appears encapsulated and composed of polygonal cells with trabecular or ribbon-like proliferation (HE 5 X). B) At immunohistochemistry, neoplastic cells showed an intense diffuse staining for glucagon (Anti-glucagon antibody 5 X)[9]

References

  1. ↑ Glucagonoma. Wikipedia. https://en.wikipedia.org/wiki/Glucagonoma. Accessed on October 13, 2015.
  2. ↑ Koike N, Hatori T, Imaizumi T; et al. (2003). "Malignant glucagonoma of the pancreas diagnoses through anemia and diabetes mellitus". Journal of hepato-biliary-pancreatic surgery. 10 (1): 101–5. PMID 12918465.
  3. ↑ Necrolytic migratory erythema. Wikipedia. https://en.wikipedia.org/wiki/Necrolytic_migratory_erythema. Accessed on October 13, 2015.
  4. ↑ Mullans EA, Cohen PR (1998). "Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema". J Am Acad Dermatol. 38 (5 Pt 2): 866–73. PMID 9591806.
  5. ↑ Kheir SM, Omura EF, Grizzle WE, Herrera GA, Lee I (1986). "Histologic variation in the skin lesions of the glucagonoma syndrome". Am J Surg Pathol. 10 (7): 445–53. PMID 3014912.
  6. ↑ 6.0 6.1 6.2 6.3 Glucagonoma. Wikimedia Commons. https://commons.wikimedia.org/wiki/File:Confluent_epidermal_necrosis_-_high_mag.jpg
  7. ↑ Castro PG, de León AM, Trancón JG, Martínez PA, Alvarez Pérez JA, Fernández Fernández JC; et al. (2011). "Glucagonoma syndrome: a case report". J Med Case Rep. 5: 402. doi:10.1186/1752-1947-5-402. PMC 3171381. PMID 21859461.
  8. ↑ 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.
  9. ↑ 9.0 9.1 9.2 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.


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