Tumour lies within the carotid sheath and so can be moved side to side but not up or down
About
- Tumour from chaemoreceptors at the bifurcation of the carotid artery.
- Has been called a glomus tumour and chaemodectoma or paraganglioma.
Aetiology
- Occur at bifurcation of the common carotid artery
- It can cause the bifurcation to splay
- Tumour can invade base of skull
- It is a rich vascular tumour
Histology
- Chief cells with clear cytoplasm and a round nucleus
- All enclosed in a fibrous tissue framework.
Risk factors
- Commoner in those living at altitude possibly due to hypoxia
- COPD, Cyanotic congenital heart disease
- Familial types exist and cause 7-10%
Rare associations
- Multiple endocrine neoplasia: MEN IIa and MEN IIb
- Tuberous sclerosis complex (TS)
- Neurofibromatosis type 1 (NF1)
- Von Hippel-Lindau disease (vHL)
Clinical
- Pulsatile swelling at medial border of sternomastoid
- Usually presents in middle age
- Tumour can be moved side to side but not up or down
- Swelling mobile laterally and Carotid bruit may be audible
- Typically asymptomatic in most just a lateral neck mass
- They may rarely release catecholamines.
Differentials
- Lymph node
- Glomus vagal tumour
- Anterior triangle masses
Investigations
- CT with contrast and MRI imaging demonstrate the tumour .
- Angiography shows a splaying of the carotid bifurcation (lyre sign).
Management
- Surgical excision may be performed especially in young healthy patients.
- Alternatives are embolization or gamma knife irradiation.
- In elderly patients a more conservative approach may be indicated
- Malignant transformation is encountered in about 10-20%
References
Revisions