phaeochromocytoma Flashcards
what is it
a catecholamine secreting tumour arisen in the adrenal medullae
who gets them
occurs every 0.5 and 2 /1000 patients with hypertension and often presents in 30s and 50s
symptoms
episodic hypertension
anxiety
weight loss
fatigue
palpitations
sweating
headaches
flushing
pyrexia
dyspnoea
abdominal pain
signs
HPT
postural hypotension
tremor
hypertensive retinopathy
what can make the signs and symptoms worse
stress
exercise
surgery
straining and various drugs ie Bbs
anaesthetic agents and opiates
ivx
-catecholamine excess using tests
> plasma metanephrines, followed by urinary metanephrines
24 hour urinary catecholamines
adrenal imaging
CT chest abdomen and pelvis imaging followed by MRI
management
definitive treatment is removal ie surgical excision
pre-operatively = alpha blockade with phenoxybenzamine (non-selective) 1st followed by Bbs
*high circulating catecholamine levels stimulate alpha receptors on blood vessels and cause vasoconstriction
fluid and/or blood replacement
careful anaesthetic assessment
classical presentation of phaeochromocytoma
high BP
headache
sweating
anxiety in a young patient
what does it look like on ct
metadrenalines > catecholamines T/F ?
T
how to identify source of catecholamine excess
MRI
MIBG
Pet
If phaochromocytoma suspected - what drugs should be discontinued before continuing with investigations
Anti hypertensive drugs - with other ‘ganglion-blocking’ anti hypertensives interferes with the catecholamine system
Adrenaline excess - drug
Phenoxybenzamine
What the fuck is Multiple endocrine neoplasia Type 2A
Presence of 2 + specific endocrine tumours
Short stature > ‘marfanoid’ habitus which excludes MEN type 2B
