Introduction
Phaeochromocytoma is a rare catecholamine secreting tumour.
Epidemiology
- Incidence: 1.00 cases per 100,000 person-years
- Peak incidence: 40-50 years
- Sex ratio: 1:1
| Condition | Relative incidence |
|---|---|
| Fibromuscular dysplasia | 10.00 |
| Phaeochromocytoma | 1 |
| <1 | 1-5 | 6+ | 16+ | 30+ | 40+ | 50+ | 60+ | 70+ | 80+ |
Pathophysiology
bout 10% are familial and may be associated with MEN type II, neurofibromatosis and von Hippel-Lindau syndrome
Basics
Basics
- bilateral in 10%
- malignant in 10%
- extra-adrenal in 10% (most common site = organ of Zuckerkandl, adjacent to the bifurcation of the aorta)
Clinical features
Features are typically episodic
- hypertension (around 90% of cases, may be sustained)
- headaches
- palpitations
- sweating
- anxiety
Investigations
- 24 hr urinary collection of metanephrines (sensitivity 97%*)
- this has replaced a 24 hr urinary collection of catecholamines (sensitivity 86%)
*BMJ 2012; 344 doi: http://dx.doi.org/10.1136/bmj.e1042 (Published 20 February 2012)
Management
Surgery is the definitive management. The patient must first however be stabilized with medical management:
- alpha-blocker (e.g. phenoxybenzamine), given before a
- beta-blocker (e.g. propranolol)