Phaeochromocytoma Flashcards
1
Q
def
A
catacholamine-producing tumours
- 90% from chromaffin cells of adrenal medulla
- 10% are extra-adrenal
- 10% are bilateral
- 10% are malignant
2
Q
what are extra-adrenal phaeochromocytomas also referred as
A
paragangliomas
3
Q
what are catecholamines
A
dopamne
adrenaline
noradrenaline
4
Q
aetiology
A
can be sporadic
genetic element in some patients
-MEN 2a, von Hippel-Lindau syndrome
5
Q
epi
A
very rare in hypertensive patients
6
Q
history
A
paroxysmal episodes of headache & sweating cardio/resp -palpitations, chest pain, dyspnoea GI -epigastric pain -nausea -constipation neuro -weakness -anxiety -tremor
7
Q
examination
A
HTN v common -2/3 sustained -1/3 paroxysmal postural hypotension secondary to reduced plasma volume pallor tachycardia fever weight loss
8
Q
investigations
A
1 24h urine collection to measure catecholamines
-certain drugs may increase catecholamines such as tricyclic antidepressants
2 plasma free metanephrines for patients at high risk
-very sensitive test
3 tumour localisation with CT/MRI
4 123I-MIBG scintigraphy for large phaeochromocytomas or paragangliomas
5 screen for associated conditions
-MEN 2a: serum calcium & calcitonin
-VHL: opthalmoscopy, MRI posterior fossa & renal USS
