Paraganglioma pathophysiology: Difference between revisions
No edit summary |
No edit summary |
||
| Line 2: | Line 2: | ||
{{Paraganglioma}} | {{Paraganglioma}} | ||
{{CMG}} | {{CMG}} {{AE}} {{AAM}} | ||
==Overview== | |||
==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 are found | 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 are 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 a part of the paraganglion system 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. | ||
==Gross Pathology== | |||
The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly [[vascular tumor]]s and may have a deep red color. | The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly [[vascular tumor]]s and may have a deep red color. | ||
==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 cells. | 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 cells. | ||
With [[immunohistochemistry]], the chief cells located in the cell balls are positive for [[chromogranin]], [[synaptophysin]], neuron specific enolase, [[serotonin]] and [[neurofilament]]; they are [[S-100 protein]] negative. The sustentacular cells are S-100 positive and focally positive for glial fibrillary acid protein. By [[histochemistry]], the paraganglioma cells are argyrophilic, [[periodic acid Schiff]] negative, [[mucicarmine]] negative, and [[argentaffin]] negative. | With [[immunohistochemistry]], the chief cells located in the cell balls are positive for [[chromogranin]], [[synaptophysin]], neuron specific enolase, [[serotonin]] and [[neurofilament]]; they are [[S-100 protein]] negative. The sustentacular cells are S-100 positive and focally positive for glial fibrillary acid protein. By [[histochemistry]], the paraganglioma cells are argyrophilic, [[periodic acid Schiff]] negative, [[mucicarmine]] negative, and [[argentaffin]] negative. | ||
Revision as of 14:41, 28 August 2015
|
Paraganglioma Microchapters |
|
Diagnosis |
|---|
|
Treatment |
|
Case Studies |
|
Paraganglioma pathophysiology On the Web |
|
American Roentgen Ray Society Images of Paraganglioma pathophysiology |
|
Risk calculators and risk factors for Paraganglioma pathophysiology |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
Overview
Pathophysiology
Paragangliomas arise from the glomus cells, which are special chemoreceptors located along blood vessels that have a role in regulating blood pressure and blood flow. The main concentration of glomus cells are found in the carotid body (located in the upper neck at the branching of the common carotid artery), and the aortic bodies (located near the aortic arch). The glomus cells are a part of the paraganglion system 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 pheochromocytomas. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.
Gross Pathology
The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm to rubbery consistency. They are highly vascular tumors and may have a deep red color.
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 cells.
With immunohistochemistry, the chief cells located in the cell balls are positive for chromogranin, synaptophysin, neuron specific enolase, serotonin and neurofilament; they are S-100 protein negative. The sustentacular cells are S-100 positive and focally positive for glial fibrillary acid protein. By histochemistry, the paraganglioma cells are argyrophilic, periodic acid Schiff negative, mucicarmine negative, and argentaffin negative.