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__NOTOC__
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
{{Paraganglioma}}
{{Paraganglioma}}
{{CMG}} {{AE}} {{AAM}}


{{Infobox_Disease
{{SK}} Chemodectoma; Paraganglioma of the spine; Pulmonary paraganglioma; Carotid paraganglioma; Vagal paraganglioma
| Name          = {{PAGENAME}}
==[[Paraganglioma overview|Overview]]==
| Image          =
| Caption        =
| DiseasesDB    = 33480
| ICD10          =
| ICD9          =  
| ICDO          = 8680-8700
| OMIM          =  
| MedlinePlus    =  
| MeshID        =
}}
{{CMG}}


==[[Paraganglioma overview|Overview]]==
==[[Paraganglioma historical perspective|Historical Perspective]]==


==Inheritance==
==[[Paraganglioma classification|Classification]]==
Familial paragangliomas account for approx. 25% of cases, are often multiple and bilateral, and occur at an earlier age.  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 (of abdomen and thorax), although there is considerable overlap in the types of tumors associated with SDHB and SDHD gene mutations.


==[[Paraganglioma pathophysiology|Pathophysiology]]==
==[[Paraganglioma pathophysiology|Pathophysiology]]==
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.
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. 


By light microscopy, the differential diagnosis includes related neuroendocrine tumors, such as [[carcinoid tumor]], neuroendocrine carcinoma, and medullary carcinoma of the thyroid; middle ear [[adenoma]]; and meningioma.
==[[Paraganglioma causes|Causes]]==


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.
==[[Paraganglioma differential diagnosis|Differentiating Paraganglioma from other Diseases]]==


==[[Paraganglioma history and symptoms|History & Symptoms]]==
==[[Paraganglioma epidemiology and demographics|Epidemiology and Demographics]]==
Paragangliomas are described by their site of origin and are often given special names:


* '''Carotid paraganglioma''' ([[carotid body]] tumor): Is the most common of the head and neck paragangliomas.  It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the [[vagus nerve]] and [[hypoglossal nerve]]. 
==[[Paraganglioma risk factors|Risk Factors]]==


* '''Glomus tympanicum''' and '''Glomus jugulare''': Both commonly present as a middle ear mass resulting in [[tinnitus]] (in 80%) and hearing loss (in 60%).  The cranial nerves of the [[jugular foramen]] may be compressed, resulting swallowing difficulty.  These patients present with a reddish bulge behind an intact ear drum.  This condition is also known as the "Red drum".  On application of pressure to the external ear canal with the help of a pneumatic ear speculum the mass could be seen to blanch.  This sign is known as "Brown's sign".
==[[Paraganglioma screening|Screening]]==


* '''Vagal paragangliomas''': These are the least common of the head and neck paragangliomas.  They usually present as a painless neck mass, but may result in [[dysphagia]] and hoarseness.
==[[Paraganglioma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


* '''Other sites''': Rare sites of involvement are the [[larynx]], nasal cavity, paranasal sinuses, [[thyroid]] gland, and the [[thoracic inlet]], as well as the bladder in extremely rare cases.
==Diagnosis==
[[Paraganglioma diagnostic study of choice|Diagnostic study of choice]] | [[Paraganglioma history and symptoms|History and Symptoms]] | [[Paraganglioma physical examination|Physical Examination]] | [[Paraganglioma laboratory findings|Laboratory Findings]] | [[Paraganglioma electrocardiogram|Electrocardiogram]] | [[Paraganglioma x ray|X-Ray Findings]] | [[Paraganglioma echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Paraganglioma CT scan|CT-Scan Findings]] | [[Paraganglioma MRI|MRI Findings]] | [[Paraganglioma other imaging findings|Other Imaging Findings]] | [[Paraganglioma other diagnostic studies|Other Diagnostic Studies]]


==[[Paraganglioma medical therapy|Medical Therapy]]==
==Treatment==
The main treatment modalities are surgery, [[embolization]] and [[radiotherapy]].
[[Paraganglioma medical therapy|Medical Therapy]] | [[Paraganglioma surgery|Surgery]] | [[Paraganglioma primary prevention|Primary Prevention]] | [[Paraganglioma secondary prevention|Secondary Prevention]] | [[Paraganglioma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Paraganglioma future or investigational therapies|Future or Investigational Therapies]]


==References==
==Case Studies==
{{reflist|2}}
[[Paraganglioma case study one|Case #1]]
{{Epithelial neoplasms}}


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Latest revision as of 06:35, 28 July 2020

For patient information click here

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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

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Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]

Synonyms and keywords: Chemodectoma; Paraganglioma of the spine; Pulmonary paraganglioma; Carotid paraganglioma; Vagal paraganglioma

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 Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | 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

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