Paraganglioma pathophysiology: Difference between revisions

Jump to navigation Jump to search
Line 15: Line 15:
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 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.
*[[Immunohistochemistry]]
*[[Immunohistochemistry]]
:*The chief cells are positive for [[chromogranin]], [[synaptophysin]], neuron specific enolase, [[serotonin]] and [[neurofilament]]; they are [[S-100 protein]] negative.  
:*In paraganglioma the chief cells are positive for [[chromogranin]], [[synaptophysin]], neuron specific enolase, [[serotonin]] and [[neurofilament]]; they are [[S-100 protein]] negative.  


*[[Histochemistry]]
*[[Histochemistry]]

Revision as of 16:09, 14 September 2015

Paraganglioma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paraganglioma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT Scan

MRI

Echocardiography and Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Paraganglioma pathophysiology On the Web

Most recent articles

cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Paraganglioma pathophysiology

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Paraganglioma pathophysiology

CDC on Paraganglioma pathophysiology

Paraganglioma pathophysiology in the news

Blogs on Paraganglioma pathophysiology

Directions to Hospitals Treating Paraganglioma

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

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 cells.

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 is 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 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, they are closely related to pheochromocytomas. Although all paragangliomas contain neurosecretory granules, only about 1-3% have clinical evidence of oversecretion.

Mutations of the genes SDHD (previously known as PGL1), PGL2, and 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.

Gross Pathology

The paragangliomas appear grossly as sharply circumscribed polypoid masses and they have a firm or 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.

References

Template:Epithelial neoplasms

Template:WikiDoc Sources