Difference between revisions of "Paraganglioma"
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'''Paraganglioma''' is a rare tumour. | '''Paraganglioma''' is a rare tumour arising from the paraganglion. | ||
==General== | ==General== | ||
* | *Definition: tumour of paraganglion. | ||
**Can be sympathetic or parasympathetic. | |||
*Head & neck most common site - after abdomen. | *Most common paraganglioma = pheochromocytoma.<ref name=Ref_EP_327>{{Ref EP|327}}</ref> | ||
**Head & neck most common site - after abdomen. | |||
*Carotid body tumour = paraganglioma of carotid body. | |||
==Epidemiology== | ==Epidemiology== | ||
*Very rare | *Very rare | ||
*Rarely malignant | *Rarely malignant | ||
*Familial syndromes assoc. with paragangliomas.<ref>EP | *Familial syndromes assoc. with paragangliomas.<ref name=Ref_EP328>{{Ref EP|328}}</ref> | ||
**[[von Hippel Lindau]]. | **[[von Hippel Lindau]]. | ||
**Hereditary paragangliomatosis. | **Hereditary paragangliomatosis. | ||
**Neurofibromatosis type 1 (von Recklinghausen disease). | **[[Neurofibromatosis]] type 1 (von Recklinghausen disease). | ||
**MEN 2A. | **[[MEN 2A]]. | ||
**MEN 2B. | **[[MEN 2B]]. | ||
==Clinical== | ==Clinical== | ||
*10% bilateral, multiple, familial, pediatric and malignant<ref>EP | *10% bilateral, multiple, familial, pediatric and malignant.<ref name=Ref_EP327>{{Ref EP|327}}</ref> | ||
==Microscopic== | ==Microscopic== | ||
Features:<ref>EP | Features:<ref>{{Ref EP|329-332}}</ref> | ||
*Resembles pheochromocytoma | *Resembles pheochromocytoma | ||
**Zellballen (literally: "cell balls") - nests of cells. | **Zellballen (literally: "cell balls") - nests of cells. | ||
| Line 28: | Line 29: | ||
==IHC== | ==IHC== | ||
Features:<ref>EP | Features:<ref>{{Ref EP|335}}</ref> | ||
*Chromogranin +ve. | *Chromogranin +ve. | ||
*Synaptophysin +ve. | *Synaptophysin +ve. | ||
| Line 38: | Line 39: | ||
==See also== | ==See also== | ||
*[[Adrenal gland]]. | *[[Adrenal gland]]. | ||
*[[Head and neck pathology]]. | |||
==References== | ==References== | ||
Revision as of 01:10, 6 December 2010
Paraganglioma is a rare tumour arising from the paraganglion.
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).
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.