Difference between revisions of "Paraganglioma"

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*[[MEN 2A]].
*[[MEN 2A]].
*[[MEN 2B]].
*[[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>
*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>


Other associations - not proven to be genetic:
Other associations - not proven to be genetic:

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]

Other associations - not proven to be genetic:

Clinical

  • 10% bilateral, multiple, familial, pediatric and malignant.[4]

Gross

  • Dusky colour.

Note:

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:

Images:

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

  1. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  2. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 328. ISBN 978-0443066856.
  3. 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.
  4. Thompson, Lester D. R. (2006). Endocrine Pathology: A Volume in Foundations in Diagnostic Pathology Series (1st ed.). Churchill Livingstone. pp. 327. ISBN 978-0443066856.
  5. 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.
  6. 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. 7.0 7.1 URL: http://path.upmc.edu/cases/case408.html. Accessed on: 16 January 2012.