Phaeochromocytoma

Description

General Measures

Medicine Treatment

Referral

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ICD-10 Code: 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 phaeochromocytoma
  • A familial syndrome that predisposes to catecholamine-secreting tumors (e.g. multiple endocrine neoplasia type 2 [MEN2], neurofibromatosis type 1 [NF1], or even 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.

LoE:III⁴⁰

• Calcium channel blockers may be added, e.g.:
  • Amlodipine, oral, 5–10 mg daily

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Note
Do not give patients diuretic therapy unless pulmonary oedema is present.
β-blockers must be used with extreme caution in the management of phaechromocytoma, and only after adequate alpha blockade

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LoE:III⁴¹

REFERRAL

• All patients.

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Standard Treatment Guidelines and Essential Medicines List for South Africa, Hospital Level, Adults 2024 Edition