Phaeochromocytomas are a rare type of catecholamine-producing neuroendocrine tumours.
A 30-year-old man complains of recurrent headaches, sweating and palpitations. On examination, the patient is hypertensive (180+systolic).
The vast majority arise from chromaffin cells in the adrenal medulla.
The remainder arise from chromaffin cells in paraganglion cells.
Up to 95% are benign, the remainder are malignant.
90% of cases are sporadic!
Characteristically, symptoms are paroxysmal and are precipitated by abdominal exertion i.e. heavy lifting or performing the Valsalva manoeuvre.
Almost 90% of patients have hypertension.
*some patients may be asymptomatic and the tumour is picked up incidentally!*
1st line - 24-hour urinary fractionated metanephrines or plasma metanephrine levels
If a plasma metanephrine sample is used - it must be taken while the patient is supine.
If there is biochemical evidence: contrast-enhanced CT scan of the abdomen should be ordered.
This section can be divided into perioperative and surgical management.
Blood pressure must be normalised before the surgery.
*if beta-blockers are given first it may precipitate a hypertensive crisis due to unopposed alpha-adrenergic stimulation.
1st line - laparoscopic adrenalectomy
Patients may be followed up with serial blood tests and imaging if the risk of recurrence is high, like in hereditary syndromes.
Diagnostics and management