Paraganglioma pathophysiology: Difference between revisions

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{{Paraganglioma}}
{{Paraganglioma}}


{{CMG}} {{AE}} {{AAM}}
{{CMG}}; {{AE}}  
==Overview==
==Overview==
On gross pathology, sharply circumscribed polypoid red vascular masses with firm to rubbery in consistency are characteristic of paragangliomas. On microscopic inspection, the tumor cells are readily recognized. Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen. These cell balls are separated by fibrovascular [[stroma]] and surrounded by [[sustentacular cell]]s.
PPGLs arise from chromaffin cells in the adrenal medulla or in extra-adrenal neural ganglia. PPGLs are related to several hereditary syndromes and many of the sporadic cases present germline mutations. Chromaffin cells are involved in the production of catecholamines, hence, these tumors can be biochemically active.
 
==Pathophysiology==
==Pathophysiology==
Paragangliomas arise from the [[glomus cell]]s, which are special [[chemoreceptor]]s located along blood vessels that have a role in regulating blood pressure and blood flow. The main concentration of glomus cells is found in the [[carotid body]] (located in the upper neck at the branching of the [[carotid artery|common carotid artery]]), and the [[aortic body|aortic bodies]] (located near the [[aorta|aortic arch]]). The glomus cells are part of the paraganglionic nervous system that composed of the extra-adrenal [[paraganglia]] of the [[autonomic nervous system]], derived from the embryonic [[neural crest]]. Thus, paragangliomas are a type of [[neuroendocrine tumor]], and are closely related to [[pheochromocytoma]]s. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.
===Physiology===
The normal physiology of [name of process] can be understood as follows:
 
===Pathogenesis===
OR
*[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
*The progression to [disease name] usually involves the [molecular pathway].
*The pathophysiology of [disease/malignancy] depends on the histological subtype.
 
==Genetics==
Genetics play an important part in PPGL pathogenesis.  


Mutations of the genes ''[[SDHD]]'' (previously known as PGL1), ''[[PGL2]]'', and ''[[SDHC (gene)|SDHC]]'' (previously PGL3) have been identified as causing familial head and neck paragangliomas. Mutations of ''[[SDHB]]'' play an important role in familial adrenal pheochromocytoma and extra-adrenal paraganglioma (abdomen and thorax), although there is considerable overlap in the types of tumors associated with ''SDHB'' and ''SDHD gene'' mutations.
[Disease name] is transmitted in [mode of genetic transmission] pattern.
 
OR
 
Genes involved in the pathogenesis of [disease name] include:
*[Gene1]
*[Gene2]
*[Gene3]
 
OR
 
The development of [disease name] is the result of multiple genetic mutations such as:
 
*[Mutation 1]
*[Mutation 2]
*[Mutation 3]
 
==Associated Conditions==
Conditions associated with [disease name] include:
 
*[Condition 1]
*[Condition 2]
*[Condition 3]


==Gross Pathology==
==Gross Pathology==
The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm or rubbery consistency. They are highly [[vascular tumor]]s and may have a deep red color.
On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
 
==Microscopic Pathology==
==Microscopic Pathology==
On microscopic inspection, the tumor cells are readily recognized.  Individual tumor cells are polygonal to oval and are arranged in distinctive cell balls, called Zellballen.  These cell balls are separated by fibrovascular [[stroma]] and surrounded by [[sustentacular cell]]s.
On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].
*[[Immunohistochemistry]]
:*The chief cells are positive for [[chromogranin]], [[synaptophysin]], neuron specific enolase, [[serotonin]] and [[neurofilament]]; they are [[S-100 protein]] negative.  


*[[Histochemistry]]
==References==
:*The paraganglioma cells are argyrophilic, [[periodic acid Schiff]] negative, [[mucicarmine]] negative, and [[argentaffin]] negative.
{{Reflist|2}}


==References==
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{{reflist|2}}
{{WS}}
{{Epithelial neoplasms}}
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[[Category:Types of cancer]]
[[Category:Mature chapter]]

Latest revision as of 21:50, 22 February 2019

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

Overview

PPGLs arise from chromaffin cells in the adrenal medulla or in extra-adrenal neural ganglia. PPGLs are related to several hereditary syndromes and many of the sporadic cases present germline mutations. Chromaffin cells are involved in the production of catecholamines, hence, these tumors can be biochemically active.

Pathophysiology

Physiology

The normal physiology of [name of process] can be understood as follows:

Pathogenesis

OR

  • [Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
  • The progression to [disease name] usually involves the [molecular pathway].
  • The pathophysiology of [disease/malignancy] depends on the histological subtype.

Genetics

Genetics play an important part in PPGL pathogenesis.

[Disease name] is transmitted in [mode of genetic transmission] pattern.

OR

Genes involved in the pathogenesis of [disease name] include:

  • [Gene1]
  • [Gene2]
  • [Gene3]

OR

The development of [disease name] is the result of multiple genetic mutations such as:

  • [Mutation 1]
  • [Mutation 2]
  • [Mutation 3]

Associated Conditions

Conditions associated with [disease name] include:

  • [Condition 1]
  • [Condition 2]
  • [Condition 3]

Gross Pathology

On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

References

Template:WH Template:WS

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