Difference between revisions of "Paraganglioma"
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*Finely granular cytoplasm (salt-and-pepper nuclei). | *Finely granular cytoplasm (salt-and-pepper nuclei). | ||
*+/-Hemorrhage - very common. | *+/-Hemorrhage - very common. | ||
DDx: | |||
*[[Neuroendocrine tumour]] - nests surrounded by stroma/do not touch. | |||
*[[Pheochromocytoma]] - paraganglioma of the [[adrenal gland]]. | |||
Images: | Images: | ||
Revision as of 14:03, 9 March 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]
- Zellballen (literally: "cell balls") - nests of cells.
- 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.
Images:
- WC:
- www:
IHC
Features:[5]
- Chromogranin +ve.
- Synaptophysin +ve.
- S100 +/-.
- Cytokeratin -ve.
- EMA -ve.
- +ve in RCC.
EM
Features:[6]
- 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.
- ↑ 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.
- ↑ 6.0 6.1 URL: http://path.upmc.edu/cases/case408.html. Accessed on: 16 January 2012.