pheochromocytoma Flashcards
defintion
rare tumour of the adrenal medulla which secretes adrenaline and noradrenaline
remember that phaeochromocytomas occur in
hereditary neuroendocrine disorders:
- MEN2A
- MEN2B
- Von Hippel lindau Syndrome
- Neurofibromatosis type 1
always rule out pheochromocytoma in
any young person with hypertension who has no significant risk factors
classic presentation of triad
- severe hypertension
- headache
- sweating
other symptoms of pheochromocytoma
- palpitations
- tachycardia
- breathlessness
- weight loss
- anxiety/ impending doom
- postural hypotension
complications of pheochromocytoma
- left ventricular failure
- myocardial necrosis
- stroke
- shock
- paralytic ileum of bowel
pheochromocytoma is described as what tumour
the 10% tumour:
- 10% malignant
- 10% bilateral
- 10% are extra-adrenal (paraglanglioma)
- 10% associated with hyperglycaemia
- 10% occur in children
- 10% are familial
diagnosis if person is presenting acutely
measure plasma catecholamines
diagnosis if patient stable
measure 24 hour urine catecholamines
when should you start treatment
immediately if plasma catecholamines are elevated
INITIAL management of pheochromocytoma
initial therapy must always be ALPHA BLOCKADE because if you use beta blockers first you will worsen the hypertension
alpha blocker used
phenoxybenzamine
after you have alpha blocked patient then what
beta block using propranolol/atenolol or bisoprolol
after the patient is stabilised
- MRI scan to locate source of excess catecholamines
- MIBG scan
- PET scan if thought to be malignant
definitive Treatment once they have been stabilised
- laparoscopic total excision (wherever possible) if this is not possible the n tumour debuting
- if malignant surgery and chemotherapy
- genetic testing for any hereditary neuroendocrine disorders
