Paraganglioma
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).
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.