Difference between revisions of "Paraganglioma"
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'''Paraganglioma''' is a rare tumour arising from the paraganglion. | '''Paraganglioma''' is a rare tumour arising from the paraganglion. A paraganglioma arising in the [[adrenal gland]] is known as a [[pheochromocytoma]]. | ||
==General== | ==General== | ||
*Definition: tumour of paraganglion. | *Definition: tumour of paraganglion. | ||
**Can be sympathetic or parasympathetic. | **Can be sympathetic or parasympathetic. | ||
*Most common paraganglioma = pheochromocytoma.<ref name=Ref_EP_327>{{Ref EP|327}}</ref> | *Most common paraganglioma = [[pheochromocytoma]].<ref name=Ref_EP_327>{{Ref EP|327}}</ref> | ||
**Head & neck most common site - after abdomen. | **Head & neck most common site - after abdomen. | ||
*Carotid body tumour = paraganglioma of carotid body. | *Carotid body tumour = paraganglioma of carotid body. | ||
Revision as of 01:50, 5 January 2012
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
- Very rare
- Rarely malignant
- Familial syndromes assoc. with paragangliomas.[2]
- von Hippel Lindau.
- Hereditary paragangliomatosis.
- Neurofibromatosis type 1 (von Recklinghausen disease).
- MEN 2A.
- MEN 2B.
Clinical
- 10% bilateral, multiple, familial, pediatric and malignant.[3]
Microscopic
Features:[4]
- Resembles pheochromocytoma
- Zellballen (literally: "cell balls") - nests of cells.
- Fibrovascular septae.
- Finely granular cytoplasm (salt-and-pepper nuclei).
Images:
- Paraganglioma - intermed. mag. (WC).
- Paraganglioma - high mag. (WC).
- Pheochromocytoma - high mag. (WC).
IHC
Features:[5]
- Chromogranin +ve.
- Synaptophysin +ve.
- S100 +/-.
- Cytokeratin -ve.
- EMA -ve.
- +ve in RCC.
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.
- ↑ 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.