Phaeochromocytoma is a neuroendocrine tumour of the medulla of the adrenal glands. It secretes large quantities of the catecholamines noradrenaline, and to a lesser extent, adrenaline.
Rule of 10: 10% familial, 10% bilateral, 10% extra-renal, 10% malignant
- Severe headaches
- Severe hypertension (eg 210/100) and orthostatic hypotension, both fluctuating
- Cardiac tachyarrythmias (eg SVTs)
- A plethora of other symptoms, including flushing+unwell+wt loss (like in hyperthyroid), panic/anxiety, sweating, pallor, hyperglycaemia, pyrexia, abdominal pain ...
- May be worsened by TCA (if you thought it was ps-somatic), beta-blockers, IV contrast
MEN 2a,2b; neurofibromatosis; Von Hippel-Lindau syndrome.
- Initial best test to rule out phaeochromocytoma: metadrenaline from blood.
- Confirmed by 3x 24-hour collection of urine catecholamines.
- CT abdo.
- Nuclear imaging: MIBG: chrommafin-seeking isotope scan, for extra-renal tumours
- Stabilise patient: ALPHA-blockers (phenoxybenzamine) BEFORE beta-blockers, to prevent hypertensive crisis from unopposed alpha-stimulation.
- Definitive Rx: surgical resection of adrenal gland.
Life-long follow up
- Malignant recurrence may present late