Paraganglioma
Paraganglioma is a rare tumour.
General
- Def'n: tumour of paraganglion (can be sympathetic or parasympathetic).
- Most common paraganglioma - pheochromocytoma[1]
- Head & neck most common site - after abdomen.
- Carotid body tumour.
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.