Paraganglioma

2017-03-29T18:25:19Z

Mario:

{{ Infobox diagnosis
| Name = {{PAGENAME}}
| Image = Paraganglioma_-_very_high_mag.jpg
| Width =
| Caption = Paraganglioma. [[H&E stain]].
| Synonyms =
| Micro = Zellballen (nests of cells), fibrovascular septae, salt-and-pepper nuclei, +/-hemorrhage (very common)
| Subtypes =
| LMDDx = [[neuroendocrine tumour]], [[pheochromocytoma]] (paraganglioma of the [[adrenal gland]]), [[gangliocytic paraganglioma]]
| Stains =
| IHC = chromogranin +ve, synaptophysin +ve, CD56 +ve
| EM =
| Molecular =
| IF =
| Gross = dusky colour
| Grossing =
| Site = abdomen (adrenal gland paraganglioma = pheochromocytoma), head and neck (carotid body tumour)
| Assdx =
| Syndromes = [[von Hippel Lindau]], hereditary paragangliomatosis, [[neurofibromatosis]] type 1 (von Recklinghausen disease), [[MEN 2A]], [[MEN 2B]], [[Carney-Stratakis syndrome]], [[Carney triad]]
| Clinicalhx =
| Signs =
| Symptoms =
| Prevalence = uncommon
| Bloodwork =
| Rads =
| Endoscopy =
| Prognosis = usually good, rarely malignant
| Other =
| ClinDDx =
| Tx =
}}
'''Paraganglioma''' is a rare tumour arising from the paraganglion. A paraganglioma arising in the [[adrenal gland]] is known as a [[pheochromocytoma]].

==General==
*Definition: tumour of paraganglion.
**Can be sympathetic or parasympathetic.
**Locations of paraganglia
***Paravertebral (retroperitoneal)
***Near the large blood vessels of the head and neck and base of skull
***Scattered in other tissues
*Most common paraganglioma = [[pheochromocytoma]].<ref name=Ref_EP_327>{{Ref EP|327}}</ref>
**Sites relate to locations of paraganglia
****Head & neck most common - neck, ear, carotid body, base of skull
****Retroperitoneal/abdomen
****Bladder

Special site names
*Carotid body tumour = paraganglioma of carotid body - very vascular - right near a major artery. Don't stick a needle in it.
*Glomus tympanicum tumor = paraganglioma of the middle ear - pulsitile tintinitis and conductive hearing loss.
*Pheochromocytoma - basically a 'paraganglioma' in the adrenal medulla

===Epidemiology===
*Rare.
*Rarely malignant.

Familial syndromes associated with paragangliomas:<ref name=Ref_EP328>{{Ref EP|328}}</ref>
*[[von Hippel Lindau]].
*Hereditary paragangliomatosis.
*[[Neurofibromatosis]] type 1 (von Recklinghausen disease).
*[[MEN 2A]].
*[[MEN 2B]].
*[[Carney-Stratakis syndrome]] - [[GIST]]s and paraganglioma.<ref>{{Cite journal | last1 = Blay | first1 = JY. | last2 = Blomqvist | first2 = C. | last3 = Bonvalot | first3 = S. | last4 = Boukovinas | first4 = I. | last5 = Casali | first5 = PG. | last6 = De Alava | first6 = E. | last7 = Dei Tos | first7 = AP. | last8 = Dirksen | first8 = U. | last9 = Duffaud | first9 = F. | title = Gastrointestinal stromal tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. | journal = Ann Oncol | volume = 23 Suppl 7 | issue = | pages = vii49-55 | month = Oct | year = 2012 | doi = 10.1093/annonc/mds252 | PMID = 22997454 | url = http://annonc.oxfordjournals.org/content/23/suppl_7/vii49.full }}</ref>
*[[Succinate dehydrogenase|SDH]] mutation associated (SDHB, SDHC and SDHD).<ref name=pmid24523625>{{Cite journal | last1 = Lefebvre | first1 = M. | last2 = Foulkes | first2 = WD. | title = Pheochromocytoma and paraganglioma syndromes: genetics and management update. | journal = Curr Oncol | volume = 21 | issue = 1 | pages = e8-e17 | month = Feb | year = 2014 | doi = 10.3747/co.21.1579 | PMID = 24523625 }}</ref>

Other associations - not proven to be genetic:
*[[Carney triad]].

===Clinical===
*10% bilateral, multiple, familial, pediatric and malignant.<ref name=Ref_EP327>{{Ref EP|327}}</ref>
**''Not'' quite true... more than 10% are familial - see ''[[pheochromocytoma]]'' article.

==Gross==
*Dusky colour.

Note:
*''Pheo'' (in [[pheochromocytoma]]) is ''dusky''; ''chromo'' is ''colour''.

Image:
*[http://commons.wikimedia.org/wiki/File:Mediastinal_paraganglioma.jpg Mediastinal paraganglioma (WC/AFIP)].

==Microscopic==
Features:<ref>{{Ref EP|329-332}}</ref>
*Zellballen - nests of cells - '''key low power feature'''.
**Zellballen is "cell balls" in German.
*Fibrovascular septae and sustentacular cells (structural support cell).
*Finely granular cytoplasm (salt-and-pepper nuclei).
*+/-Hemorrhage - very common.

DDx:
*[[Neuroendocrine tumour]] - nests surrounded by stroma/do not touch.
*[[Pheochromocytoma]] - paraganglioma of the [[adrenal gland]].
*[[Gangliocytic paraganglioma]] - has schwannian component and ganglion cells, usu. [[duodenum]].

===Images===
Carotid body tumour:
<gallery>
Image:Carotid_body_tumour_2_intermed_mag.jpg | Paraganglioma - intermed. mag. (WC)
Image:Carotid_body_tumour_2_high_mag.jpg | Paraganglioma - high mag. (WC)
Image:Neck Paraganglioma HP CTR (2).jpg|Neck - Paraganglioma - nice Zeballen (SKB)
Image:Neck Paraganglioma CarotidBody MP PA.JPG|Neck Paraganglioma - Carotid Body Tumor (SKB)
Image:Neck Paraganglioma CarotidBody HP PA.JPG|Neck - Paraganglioma - Carotid Body Tumor (SKB)
</gallery>
Duodenal paraganglioma - uncommon location:
<gallery>
Image:Paraganglioma_-_low_mag.jpg | Paraganglioma - low mag. (WC)
Image:Paraganglioma_-_very_high_mag.jpg | Paraganglioma - very high mag. (WC)
Image:Paraganglioma_-_chromo_-_intermed_mag.jpg | Paraganglioma - chromogranin A - intermed. mag. (WC)
Image:Paraganglioma_-_s100_-_very_high_mag.jpg | Paraganglioma - S100 - very high mag. (WC)
</gallery>
Retroperitoneal paraganglioma
<gallery>
Image:Retroperitoneum Paraganglioma 2 MP PA.JPG|Retroperitoneum - Paraganglioma - Prominent vascular component (SKB)
Image:Retroperitoneum Paraganglioma 2 HP PA.JPG|Retroperitoneum - Paraganglioma (SKB)
Image:Retroperitoneum Paraganglioma HP PA.JPG|Retroperitoneum - Paraganglioma - florid atypia (SKB)
Image:Retroperitoneum Paraganglioma MP CTR.jpg|Retroperitoneum - Paraganglioma - large nests (SKB)
</gallery>
Ear paraganglioma "Glomus Tympanicum"
<gallery>
Image:Ear Paraganglioma GlomusTympanicumTumor MP PA.JPG|Ear - Paraganglioma - Glomus Tympanicum Tumor (SKB)
Image:Ear Paraganglioma GlomusTympanicumTumor HP 2 PA.JPG|Ear - Paraganglioma - Glomus Tympanicum Tumor (SKB)
</gallery>
Bladder
<gallery>
Image:Bladder Paraganglioma PA DSCN4717.JPG|Bladder - Paraganglioma - Presented as micturation syncope (SKB)
</gallery>
Other:
<gallery>
Image:Pheochromocytoma_high_mag.jpg | Pheochromocytoma - high mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case523.html Paraganglioma with gangliocytic differentiation - several images (upmc.edu)].

==IHC==
Features:<ref name=Ref_EP335>{{Ref EP|335}}</ref>
*Chromogranin +ve.
*Synaptophysin +ve.
*S100 +ve/-ve (+ve in sustentacular cells, not tumor cells)
*Cytokeratin -ve.
*[[EMA]] -ve.
**+ve in [[renal cell carcinoma|RCC]].

==EM==
Features:<ref name=em_stuff>URL: [http://path.upmc.edu/cases/case408.html http://path.upmc.edu/cases/case408.html]. Accessed on: 16 January 2012.</ref>
*Neurosecretory granules.
**Electron dense core.
**Typically perinuclear location.

Image:
*[http://path.upmc.edu/cases/case408/images/fig14.jpg Neurosecretory granules (upmc.edu)].<ref name=em_stuff>URL: [http://path.upmc.edu/cases/case408.html http://path.upmc.edu/cases/case408.html]. Accessed on: 16 January 2012.</ref>

==Sign out==
<pre>
SOFT TISSUE, LEFT/RIGHT CAROTID BODY, EXCISION:
- PARAGANGLIOMA (SIZE IN CM).
- NEGATIVE RESECTION MARGIN.
</pre>

==See also==
*[[Adrenal gland]].
*[[Head and neck pathology]].

==References==
{{reflist|2}}

[[Category:Weird stuff]]
[[Category:Diagnosis]]

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Paraganglioma