Hepatic hemangioma pathophysiology: Difference between revisions

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{{Hepatic hemangioma}}
{{Hepatic hemangioma}}
{{CMG}}
{{CMG}};{{AE}}{{NM}}
==Overview==
Development of hepatic hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.<ref name="PapafragkakisMoehlen2011">{{cite journal|last1=Papafragkakis|first1=Haris|last2=Moehlen|first2=Martin|last3=Garcia-Buitrago|first3=Monica T.|last4=Madrazo|first4=Beatrice|last5=Island|first5=Eddie|last6=Martin|first6=Paul|title=A Case of a Ruptured Sclerosing Liver Hemangioma|journal=International Journal of Hepatology|volume=2011|year=2011|pages=1–5|issn=2090-3456|doi=10.4061/2011/942360}}</ref> Hepatic hemangioma may be associated with [[Kasabach-Merritt syndrome]], [[hereditary hemorrhagic telangiectasia]], hepatic arterio-portal shunts, extra hepatic hemangiomata, [[hemolytic anemia]], [[focal nodular hyperplasia]].<ref name=radiopeadia>Associations of hepatic hemangioma. Dr Yuranga Weerakkody et al. Radiopaedia (2015). http://radiopaedia.org/articles/hepatic-haemangioma-3. Accessed on November 7, 2015</ref> On gross pathology, variable in size, well circumscribed, and classically subcapsular are findings of hepatic hemangioma.<ref name=Librepthology>Gross pathology of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref> On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.<ref name=Librepathology>Microscopic features of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref>
==Pathophysiology==
==Pathogenesis==
The pathogenesis of hepatic hemangiomas has not been elucidated, but there are two competing theories.
===First theory===
*The first theory supports the notion that hepatic hemangioma may be the result of overexpression of angiogenic factors and downregulation of inhibitors of angiogenesis.
:*Overexpression of angiographic factors, such as:<ref name="PapafragkakisMoehlen2011">{{cite journal|last1=Papafragkakis|first1=Haris|last2=Moehlen|first2=Martin|last3=Garcia-Buitrago|first3=Monica T.|last4=Madrazo|first4=Beatrice|last5=Island|first5=Eddie|last6=Martin|first6=Paul|title=A Case of a Ruptured Sclerosing Liver Hemangioma|journal=International Journal of Hepatology|volume=2011|year=2011|pages=1–5|issn=2090-3456|doi=10.4061/2011/942360}}</ref>
::*[[Vascular endothelial growth factor]]
::*[[Basic fibroblast growth factor]]
::*[[Metalloproteinases]]
:*Downregulation of inhibitors of angiogenesis, such as:<ref name="PapafragkakisMoehlen2011">{{cite journal|last1=Papafragkakis|first1=Haris|last2=Moehlen|first2=Martin|last3=Garcia-Buitrago|first3=Monica T.|last4=Madrazo|first4=Beatrice|last5=Island|first5=Eddie|last6=Martin|first6=Paul|title=A Case of a Ruptured Sclerosing Liver Hemangioma|journal=International Journal of Hepatology|volume=2011|year=2011|pages=1–5|issn=2090-3456|doi=10.4061/2011/942360}}</ref>
::*[[Metalloproteinase|Tissue inhibitor of metalloproteinase-I]]
 
===Second theory===
*The second theory suggests that the presence of liver hemangiomas involves a genetic background of mutations.<ref name="PapafragkakisMoehlen2011">{{cite journal|last1=Papafragkakis|first1=Haris|last2=Moehlen|first2=Martin|last3=Garcia-Buitrago|first3=Monica T.|last4=Madrazo|first4=Beatrice|last5=Island|first5=Eddie|last6=Martin|first6=Paul|title=A Case of a Ruptured Sclerosing Liver Hemangioma|journal=International Journal of Hepatology|volume=2011|year=2011|pages=1–5|issn=2090-3456|doi=10.4061/2011/942360}}</ref>
:*[[Metalloproteinases]] accumulate in the endoplasmic reticulum of the tumor cells. Accumulation may result in the following:
::*Self-digestion
::*Vacuole formation
*Cavernous hemangioma cell to downregulate Derlin-1.
:*Derlin-1 is a protein encoded by [[DERL1]]. When overexpressed, derlin-1 protein induces the dilated endoplasmic reticulum to return to its normal size.


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==Associated Conditions==
==Pathophysiology==
Hepatic hemangioma may be associated with:<ref name=radiopeadia>Associations of hepatic hemangioma. Dr Yuranga Weerakkody et al. Radiopaedia (2015). http://radiopaedia.org/articles/hepatic-haemangioma-3. Accessed on November 7, 2015</ref>
*[[Kasabach-Merritt syndrome]]
*[[Hereditary hemorrhagic telangiectasia]] ([[Hereditary hemorrhagic telangiectasia|Osler-Weber-Rendu disease]])
*Hepatic arterio-portal shunts
*Extra hepatic hemangiomata
*[[Hemolytic anemia]]
*[[DIC|Consumptive coagulopathy]]
*[[Focal nodular hyperplasia]]


* They arise from the endothelial cells that line the blood vessels and consists of multiple, large vascular channels lined by a single layer of endothelial cells and supported by collagenous walls.
==Gross Pathology==
* They may be associated with [[focal nodular hyperplasia]].  
On gross pathology, a well circumscribed, subcapsular tumor may be suggestive of hepatic hemangioma.<ref name=Librepthology>Gross pathology of hepatic hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref>
* May be associated with [[Kasabach-Merritt syndrome]], [[hemolytic anemia]] and consumptive coagulopathy.


==Microscopic Pathology==
On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.<ref name=Librepathology>Microscopic features of hepatic hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref>
==Gallery==
<gallery>
Image:
Cavernous_liver_hemangioma_-_intermed_mag.jpg|Intermediate magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.<ref name=Librepathology>Image of microscopy of Hepatic hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref>
Image:
Cavernous liver hemangioma high magnification.jpg|High magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.<ref name=Librepathology>Image of microscopy of Hepatic hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015</ref>
</gallery>
==References==
==References==


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Latest revision as of 01:56, 27 November 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]

Overview

Development of hepatic hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis.[1] Hepatic hemangioma may be associated with Kasabach-Merritt syndrome, hereditary hemorrhagic telangiectasia, hepatic arterio-portal shunts, extra hepatic hemangiomata, hemolytic anemia, focal nodular hyperplasia.[2] On gross pathology, variable in size, well circumscribed, and classically subcapsular are findings of hepatic hemangioma.[3] On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.[4]

Pathophysiology

Pathogenesis

The pathogenesis of hepatic hemangiomas has not been elucidated, but there are two competing theories.

First theory

  • The first theory supports the notion that hepatic hemangioma may be the result of overexpression of angiogenic factors and downregulation of inhibitors of angiogenesis.
  • Overexpression of angiographic factors, such as:[1]
  • Downregulation of inhibitors of angiogenesis, such as:[1]

Second theory

  • The second theory suggests that the presence of liver hemangiomas involves a genetic background of mutations.[1]
  • Metalloproteinases accumulate in the endoplasmic reticulum of the tumor cells. Accumulation may result in the following:
  • Self-digestion
  • Vacuole formation
  • Cavernous hemangioma cell to downregulate Derlin-1.
  • Derlin-1 is a protein encoded by DERL1. When overexpressed, derlin-1 protein induces the dilated endoplasmic reticulum to return to its normal size.

Associated Conditions

Hepatic hemangioma may be associated with:[2]

Gross Pathology

On gross pathology, a well circumscribed, subcapsular tumor may be suggestive of hepatic hemangioma.[3]

Microscopic Pathology

On microscopic histopathological analysis channels lined by benign endothelium containing RBCs, surrounding (non-endothelial) cells without significant atypia are findings of hepatic hemangioma.[4]

Gallery

  • Intermediate magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.[4]

    Intermediate magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.[4]

  • High magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.[4]

    High magnification micrograph of a cavernous hemangioma of the liver, also hepatic cavernous hemangioma, liver hemangioma,cavernous liver hemangioma. H&E stain. No liver tissue is observed.[4]

References

  1. ↑ 1.0 1.1 1.2 1.3 Papafragkakis, Haris; Moehlen, Martin; Garcia-Buitrago, Monica T.; Madrazo, Beatrice; Island, Eddie; Martin, Paul (2011). "A Case of a Ruptured Sclerosing Liver Hemangioma". International Journal of Hepatology. 2011: 1–5. doi:10.4061/2011/942360. ISSN 2090-3456.
  2. ↑ 2.0 2.1 Associations of hepatic hemangioma. Dr Yuranga Weerakkody et al. Radiopaedia (2015). http://radiopaedia.org/articles/hepatic-haemangioma-3. Accessed on November 7, 2015
  3. ↑ 3.0 3.1 Gross pathology of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015
  4. ↑ 4.0 4.1 4.2 4.3 Microscopic features of hepatic hemangioma. Librepathology 2015. http://librepathology.org/wiki/index.php/Hemangioma_of_the_liver. Accessed on October 20, 2015
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