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Paraganglioma

Dr Charlie Chia-Tsong Hsu and Dr Frank Gaillard et al.

Paragangliomas (also called glomus tumours) are slow growing tumours arising non chromaffin paraganglion cells that are scattered throughout the body from the glomus jugulotympanicum to the bladder.

Pathology

Paragangliomas can occur anywhere in where there is a globus body and are divided according to their location:

paravertebral paraganglia :
- related to sympathetic nervous system and often (~ 50%) secrete catecholamines
- thus extra-adrenal pheochromocytomas : locations include organ of Zuckerkandl and bladder (rare)
paraganglia : related to the great vessels of the head and neck and base of skull
scattered in other tissues : bladder, ciliary ganglion, pharynx and larynx

They are sometimes referred to as glomus tumours although this gives rise to confusion with glomangioma, a tumour of the glomus body in the skin and soft tissues and should thus be avoided.

Histologically they contain nests of polygonal cells (Zellballen) enclosed by trabeculating fibrous components and sustentacular cells.