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| '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
| | {{Paraganglioma}} |
| | {{CMG}} {{AE}} {{AAM}} |
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| {{Infobox_Disease | | {{SK}} Chemodectoma; Paraganglioma of the spine; Pulmonary paraganglioma; Carotid paraganglioma; Vagal paraganglioma |
| | Name = {{PAGENAME}}
| | ==[[Paraganglioma overview|Overview]]== |
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| | DiseasesDB = 33480
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| | ICDO = 8680-8700
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| | eMedicineSubj = med
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| | eMedicineTopic = 2994
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| ==Overview== | | ==[[Paraganglioma historical perspective|Historical Perspective]]== |
| A '''paraganglioma''' is a rare [[neoplasm]] that can be found in the abdomen, thorax, and in the head and neck region. They are usually considered [[benign]] and complete surgical removal results in cure. However, in about 3% of cases they are [[malignant]] and have the ability to [[metastasis|metastasize]]. Paragangliomas are still sometimes called '''glomus tumors''' (not to be confused with [[glomus tumor]]s of the skin) and '''chemodectomas''', but paraganglioma is the currently accepted and preferred term.
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| 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 are 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.
| | ==[[Paraganglioma classification|Classification]]== |
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| Paragangliomas are found predominantly in the abdomen (85%) and the thorax (12%), and only 3% are found in the head and neck region. Most occur as single tumors. When they occur in multiple sites they are usually found as a part of a heritable syndrome such as [[multiple endocrine neoplasia]] types II-A and II-B and [[SDH]]-related mutations.
| | ==[[Paraganglioma pathophysiology|Pathophysiology]]== |
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| According to the [[World Health Organization]] classification of neuroendocrine tumors, paragangliomas are classified as having a neural cell line of origin. In the categorization proposed by Wick, the paragangliomas belong to Group II.
| | ==[[Paraganglioma causes|Causes]]== |
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| ==Inheritance== | | ==[[Paraganglioma differential diagnosis|Differentiating Paraganglioma from other Diseases]]== |
| 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.
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| ==Pathology== | | ==[[Paraganglioma epidemiology and demographics|Epidemiology and Demographics]]== |
| 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.
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| 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.
| | ==[[Paraganglioma risk factors|Risk Factors]]== |
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| 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 screening|Screening]]== |
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| 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 natural history, complications and prognosis|Natural History, Complications and Prognosis]]== |
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| ==Clinical characteristics== | | ==Diagnosis== |
| Paragangliomas are described by their site of origin and are often given special names:
| | [[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]] |
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| * '''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]].
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| * '''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".
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| * '''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.
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| * '''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.
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| ==Treatment== | | ==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]] |
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| ==References==
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| * Pellitteri PK et al. Paragangliomas of the head and neck. ''Oral Oncology'' 2004 Jul;40(6):563-75. ([http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=15063383 PMID 15063383])
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| *[http://www.accessscience.com/server-java/Arknoid/science/AS/Encyclopedia/0/04/Est_042400_frameset.html Sukhamay Lahiri, "Aortic body", in AccessScience@McGraw-Hill]
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| * [http://www.accessscience.com/server-java/Arknoid/science/AS/Encyclopedia/1/11/Est_110700_frameset.html John T. Hansen, "Carotid body", in AccessScience@McGraw-Hill]
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| * Wick MR. Neuroendocrine neoplasia. Current concepts. ''Am J Clin Pathol''. 2000 Mar;113(3):331-5. ([http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=10705811 PMID 10705811])
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| * [http://www.theotologygroup.com/skullbased/s1.html The Otology Group]
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| External Links:
| | ==Case Studies== |
| | [[Paraganglioma case study one|Case #1]] |
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| *[http://www.drtbalu.com/glomus.html (Description with pictures)]
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| [[Category:Types of cancer]] | | [[Category:Up-To-Date]] |
| [[Category:Oncology]] | | [[Category:Oncology]] |
| {{Epithelial neoplasms}}
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| | [[Category:Neurology]] |
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