Phaeochromocytoma Flashcards
What is a phaechromocytoma?
A tumour of the chromaffin cells in the adrenal glands that secretes unregulated and excessive amounts of catecholamines (adrenaline)
What cells produce adrenaline?
Chromaffin cells
Where are chromaffin cells located?
The medulla of the adrenal glands
What is adrenaline?
A catecholamine hormone that stimulates the sympathetic nervous system
In which genetic disorders are phaeochromocytomas more common?
Multiple endocrine neoplasia t2
Neurofibromatosis type 1
Von Hippel-Lindau disease
Over periods when a phaeochromocytoma is secreting adrenaline what are common clinical signs + symptoms?
Anxiety, sweating
Headache
Tremor
Palpitations
Hypertension, tachycardia
What are the initial tests for a phaeochromocytoma?
Plasma free metanephrines
24-hour urine catecholamines
What are metanephrines?
A breakdown product of adrenaline
What does a 24-hour urine catecholamines test tell you?
An idea of how much adrenaline is being secreted by a phaeochromocytoma over 24 hours
What scan(s) can be used to investigate a phaeochromocytoma?
CT
MRI
What is the definitive management of a phaeochromocytoma?
Surgical removal of the tumour
What medications are used to control the symptoms of a phaeochromocytoma?
Alpha blockers (doxazosin, phenoxybenzamine)
Beta blockers