Difference between revisions of "Hemangioma pathophysiology"

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{{CMG}};{{AE}}{{NM}}
 
{{CMG}};{{AE}}{{NM}}
 
==Overview==
 
==Overview==
 +
Development of hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis. Hemangioma may be associated with [[POEMS syndrome]] and [[Castleman disease]]. On gross pathology, spongy with vascular compartments of various sizes separated by fibrous tissue are findings of hemangioma. On microscopic histopathological analysis, channels lined by benign endothelium containing red blood cells are findings of hemangioma.
 
==Pathophysiology==
 
==Pathophysiology==
==Pathogenesis==
+
The pathogenesis of hemangiomas has not been elucidated, but there are two competing theories;
==Genetics==
+
===First theory===
 +
*The first theory supports the notion that there is overexpression of angiogenic 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|Matrix metalloproteinases]]
 +
*And there is downregulation of some 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 is 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>
 +
*Genetic errors in growth factor receptors have also been shown to affect development of hemangiomas.
 +
*[[Metalloproteinases]] can accumulate in the endoplasmic reticulum of the tumor cells causing:
 +
**Self-digestion
 +
**Vacuole formation
 +
*Cavernous hemangioma cell can downregulate Derlin-1.
 +
**[[Derlin-1|Derlin]]-1 is a protein that when overexpressed induces the dilated endoplasmic reticulum to return to its normal size.
 +
===Third theory===
 +
*The third theory suggests that hemangioma endothelial cells arise from disrupted placental tissue imbedded in fetal soft tissues during [[gestation]] or birth.<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref>
 +
*Markers of hemangiomas have been shown to coincide with those found in [[placental]] tissue.
 +
*This is further supported by the fact that they are found more commonly in infants following:<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref>
 +
**[[Chorionic villus sampling]]
 +
**[[Placenta previa]]
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**[[Preeclampsia]]
 +
 
 +
===Growth Pattern===
 +
*Hemangiomas follow a predictable course with three distinct developmental phases:<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref>
 +
**[[Proliferation]] phase
 +
**[[Quiescence]] phase
 +
**Involution phase
 +
 
 +
=====Proliferation phase=====
 +
*In most hemangiomas, eighty percent of proliferation occurs by three months of life but may last longer.<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref><ref name="Librepathology">Microscopic features of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref>
 +
*During proliferation, rapid growth can lead to exhaustion of blood supply with resulting ischemia, necrosis, ulceration, and bleeding.
 +
=====Quiescence phase=====
 +
*Following proliferation, hemangiomas enter a slower or no growth phase, known as [[quiescence]].<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref>
 +
*This phase typically lasts from nine to twelve months of age.
 +
=====Involution phase=====
 +
*The final and unique phase of the hemangioma lifecycle is involution.<ref name="RichterFriedman2012">{{cite journal|last1=Richter|first1=Gresham T.|last2=Friedman|first2=Adva B.|title=Hemangiomas and Vascular Malformations: Current Theory and Management|journal=International Journal of Pediatrics|volume=2012|year=2012|pages=1–10|issn=1687-9740|doi=10.1155/2012/645678}}</ref>
 +
*This phase is marked by graying of the overlying skin and shrinking of the deeper components.
 +
*At the final stages of involution, a fibrofatty protuberance may remain.
 +
 
 
==Associated Conditions==
 
==Associated Conditions==
 
Hemangioma may be associated with:
 
Hemangioma may be associated with:
*POEMS syndrome
+
*[[POEMS syndrome]]
*Castleman disease
+
*[[Castleman disease]]
 +
*[[PHACES syndrome]]
 +
==Gross Pathology==
 +
*Grossly hemangiomas are described as “spongy” with vascular compartments of various sizes separated by [[fibrous]] tissue.<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>
  
==Gross Pathology==
+
*Thrombi may be present and are well separated from the normal liver parenchyma despite the absence of a [[fibrous capsule]].
 
==Microscopic Pathology==
 
==Microscopic Pathology==
==Gallery==
+
On microscopic histopathological analysis channels lined by benign endothelium containing red blood cells are findings of hemangioma.<ref name="Librepathology">Microscopic features of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref>
 +
===Gallery===
 +
<gallery>
 +
Image:
 +
Capillary_hemangioma_intermediate_magnification.jpg|<sub>Intermediate magnification micrograph of a capillary hemangioma. H&E stain.<ref name=Librepathology>Image of microscopy of Hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref></sub>
 +
Image:
 +
Capillary_hemangioma_very_high_magnification.jpg|<sub>Very high magnification micrograph of a capillary hemangioma. H&E stain.<ref name=Librepathology>Image of microscopy of Hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref></sub>
 +
Image:
 +
Cavernous_hemangioma_histopathology.jpg|<sub>Histopathological image representing a cavernous hemangioma of the liver. Surgical excision of the lesion for the impending risk for rupture. H&E stain.<ref name=Librepathology>Image of microscopy of Hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref></sub>
 +
Image:
 +
Cavernous_hemangioma_histopathology2.jpg|<sub>Histopathological image reprsenting a cavernous hemangioma of the liver. H&E stain.<ref name=Librepathology>Image of microscopy of Hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref></sub>
 +
</gallery>
 +
==Immunohistochemistry==
 +
Hemangioma is demonstrated by positivity to:<ref name="Librepathology">Immunohistochemistry of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015</ref>
 +
*CD31 positive
 +
*D2-40 negative
  
 
==References==
 
==References==
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{{WH}}
 
{{WH}}
 
{{WS}}
 
{{WS}}
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[[Category:Up-To-Date]]
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[[Category:Oncology]]
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[[Category:Medicine]]
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[[Category:Vascular medicine]]
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[[Category:Surgery]]

Latest revision as of 13:13, 14 August 2019

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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 hemangioma is the result of genetic mutations, overexpression of angiogenic fators and downregulation of inhibitors of angiogenesis. Hemangioma may be associated with POEMS syndrome and Castleman disease. On gross pathology, spongy with vascular compartments of various sizes separated by fibrous tissue are findings of hemangioma. On microscopic histopathological analysis, channels lined by benign endothelium containing red blood cells are findings of hemangioma.

Pathophysiology

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

First theory

Second theory

  • The second theory is that the presence of liver hemangiomas involves a genetic background of mutations.[1]
  • Genetic errors in growth factor receptors have also been shown to affect development of hemangiomas.
  • Metalloproteinases can accumulate in the endoplasmic reticulum of the tumor cells causing:
    • Self-digestion
    • Vacuole formation
  • Cavernous hemangioma cell can downregulate Derlin-1.
    • Derlin-1 is a protein that when overexpressed induces the dilated endoplasmic reticulum to return to its normal size.

Third theory

  • The third theory suggests that hemangioma endothelial cells arise from disrupted placental tissue imbedded in fetal soft tissues during gestation or birth.[2]
  • Markers of hemangiomas have been shown to coincide with those found in placental tissue.
  • This is further supported by the fact that they are found more commonly in infants following:[2]

Growth Pattern

  • Hemangiomas follow a predictable course with three distinct developmental phases:[2]
Proliferation phase
  • In most hemangiomas, eighty percent of proliferation occurs by three months of life but may last longer.[2][3]
  • During proliferation, rapid growth can lead to exhaustion of blood supply with resulting ischemia, necrosis, ulceration, and bleeding.
Quiescence phase
  • Following proliferation, hemangiomas enter a slower or no growth phase, known as quiescence.[2]
  • This phase typically lasts from nine to twelve months of age.
Involution phase
  • The final and unique phase of the hemangioma lifecycle is involution.[2]
  • This phase is marked by graying of the overlying skin and shrinking of the deeper components.
  • At the final stages of involution, a fibrofatty protuberance may remain.

Associated Conditions

Hemangioma may be associated with:

Gross Pathology

  • Grossly hemangiomas are described as “spongy” with vascular compartments of various sizes separated by fibrous tissue.[1]
  • Thrombi may be present and are well separated from the normal liver parenchyma despite the absence of a fibrous capsule.

Microscopic Pathology

On microscopic histopathological analysis channels lined by benign endothelium containing red blood cells are findings of hemangioma.[3]

Gallery

Immunohistochemistry

Hemangioma is demonstrated by positivity to:[3]

  • CD31 positive
  • D2-40 negative

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 2.2 2.3 2.4 2.5 Richter, Gresham T.; Friedman, Adva B. (2012). "Hemangiomas and Vascular Malformations: Current Theory and Management". International Journal of Pediatrics. 2012: 1–10. doi:10.1155/2012/645678. ISSN 1687-9740.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 Microscopic features of hemangioma. Librepathology (2015). http://librepathology.org/wiki/index.php/Hemangioma. Accessed on November 12, 2015

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