Carotid body tumour
Paraganglioma of the carotid body
Aetiology
[edit | edit source]- Sporadic 85%
- Familial - VHL, NF1, MEN2, SDH
- Hyperplastic - associated with chronic hypoxia
Pathophysiology
[edit | edit source]- Paraganglioma - can be higher risk of neural crest tumours elsewhere
- Based on the carotid body - a cluster of chemoreceptors in the adventitia of the carotid bifurcation
- Highly vascular
- Senses partial pressure of oxygen in blood, and signals respiratory centre via glossopharyngeal nerve
- Tumours therefore develop in the adventitia of the medial aspect of the carotid body
- Rarely secrete catecholamines
Presentation
[edit | edit source]- Slow-growing, painless, lateral neck mass
- Local invasion or compression is a late sign
- Symptoms of catecholamines (as per phaeochromocytoma)
- Fontaine's sign - fixed vertically but mobile horizontally
Imaging:
[edit | edit source]- Lyre sign - splaying of ICA/ECA
Classification
[edit | edit source]- Shamblin's classification
- Type 1 - easily resected
- Type 2 - adherent to surrounding vessels
- Type 3 - intimately surround or encase vessels or nerves
Investigations
[edit | edit source]- 24 hour urinary catecholamines
- CT/MRI
- Angiography pre-op
- No biopsy
Management
[edit | edit source]- Observe <2cm lesions
- Resect symptomatic or larger