Difference between revisions of "Paraganglioma"
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===Epidemiology=== | ===Epidemiology=== | ||
* | *Rare. | ||
*Rarely malignant | *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 - GISTs 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> | |||
Other associations - not proven to be genetic: | |||
*[[Carney triad]]. | |||
===Clinical=== | ===Clinical=== | ||
Revision as of 04:57, 5 March 2013
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.
- Most common paraganglioma = pheochromocytoma.[1]
- Head & neck most common site - after abdomen.
- Carotid body tumour = paraganglioma of carotid body.
Epidemiology
- Rare.
- Rarely malignant.
Familial syndromes associated with paragangliomas:[2]
- von Hippel Lindau.
- Hereditary paragangliomatosis.
- Neurofibromatosis type 1 (von Recklinghausen disease).
- MEN 2A.
- MEN 2B.
- Carney-Stratakis syndrome - GISTs and paraganglioma.[3]
Other associations - not proven to be genetic:
Clinical
- 10% bilateral, multiple, familial, pediatric and malignant.[4]
Gross
- Dusky colour.
Note:
- Pheo (in pheochromocytoma) is dusky; chromo is colour.
Image:
Microscopic
Features:[5]
- Zellballen - nests of cells - key low power feature.
- Zellballen is "cell balls" in German.
- Fibrovascular septae.
- 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:
- WC:
- Carotid body tumour:
- Duodenal paraganglioma - uncommon location:
- Other:
- www:
IHC
Features:[6]
- Chromogranin +ve.
- Synaptophysin +ve.
- S100 +/-.
- Cytokeratin -ve.
- EMA -ve.
- +ve in RCC.
EM
Features:[7]
- Neurosecretory granules.
- Electron dense core.
- Typically perinuclear location.
Image:
See also
References
- ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
- ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 328. ISBN 978-0443066856.
- ↑ Blay, JY.; Blomqvist, C.; Bonvalot, S.; Boukovinas, I.; Casali, PG.; De Alava, E.; Dei Tos, AP.; Dirksen, U. et al. (Oct 2012). "Gastrointestinal stromal tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up.". Ann Oncol 23 Suppl 7: vii49-55. doi:10.1093/annonc/mds252. PMID 22997454. http://annonc.oxfordjournals.org/content/23/suppl_7/vii49.full.
- ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
- ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 329-332. ISBN 978-0443066856.
- ↑ Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 335. ISBN 978-0443066856.
- ↑ 7.0 7.1 URL: http://path.upmc.edu/cases/case408.html. Accessed on: 16 January 2012.