C74.0-1/C74.9/C79.7/D09.3/D35.0/D44.1 + (M8700/0/3/6)
DESCRIPTION
Catecholamine-secreting tumour of the adrenal medulla.
Clinical presentation
Always consider in hypertensive patients who have paroxysmal symptoms:
- headaches,
- tremor,
- GIT symptoms,
- recurrent chest discomfort,
- palpitations,
- sweating, and
- anxiety.
There is marked inter-individual variation in symptoms.
Patients may also have orthostatic changes in BP.
Diagnosis
24-hour urine acidified with HCl: normetanephrine (NMA), vanillylmandelic acid (VMA), should be ≥ twice normal for a definite diagnosis. Test is best done during a paroxysm, if possible, using at least 2 samples.
There are many drugs, foods and diseases that can falsely elevate or lower NMA/VMA levels; therefore, the clinician must interpret the results in the light of the clinical context and after having taken an accurate history.
Screen:
young hypertensive patients;
hypertensive patients with paroxysmal symptoms; and
patients with:
- The classic triad of headache, sweating, and tachycardia, whether or not they have hypertension
- a family history of a phaeochromocytoma,
- A familial syndrome that predisposes to catecholamine-secreting tumours (e.g., multiple endocrine neoplasia type 2 [MEN2], neurofibromatosis type 1 [NF1], or von Hippel-Lindau [VHL]). or
- radiologic evidence of an adrenal mass (adrenal incidentaloma) with or without hypertension.
GENERAL MEASURES
Surgical removal of the tumour.
MEDICINE TREATMENT
Once diagnosis is confirmed, initiate medication with immediate referral.
- Alpha blockers, e.g.:
- Doxazosin, oral, 4 mg daily.
- Dose increase above 8 mg daily to control blood pressure may be required.
- Calcium channel blockers may be added, e.g.:
- Amlodipine, oral, 5–10 mg daily.
Note:
- Do not give patients diuretic therapy unless pulmonary oedema is present.
- β-blockers must be used with extreme caution in the management of phaeochromocytoma, and only after adequate alpha blockade.
REFERRAL
All patients.