Pheochromocytoma: Screening and Diagnosis

  1. If pheochromocytoma is strongly suspected, the patient should be referred to a specialized hypertension center, particularly if biochemical screening tests (Supplemental Table S8) have already been found to be positive (Grade D).
  2. The following patients should be considered for screening for pheochromocytoma or paraganglioma (Grade D):
    1. Patients with paroxysmal, unexplained, labile, and/or severe (BP ≥180/110 mmHg) sustained hypertension refractory to usual antihypertensive therapy;
    2. Patients with hypertension and multiple symptoms suggestive of catecholamine excess (e.g., headaches, palpitations, sweating, panic attacks and pallor);
    3. Patients with hypertension triggered by betablockers, monoamine oxidase inhibitors, micturition, changes in abdominal pressure, surgery, or anesthesia;
    4. Patients with an incidentally discovered adrenal mass;
    5. Patients with a predisposition to hereditary causes (e.g. multiple endocrine neoplasia 2A or 2B, von Recklinghausen’s neurofibromatosis type 1, or von Hippel-Lindau disease).
    6. For patients with positive biochemical screening tests, localization of pheochromocytomas or paragangliomas should employ magnetic resonance imaging (preferable), computed tomography (if MRI unavailable), and/or iodine I-131 metaiodobenzylguanidine (MIBG) scintigraphy (Grade C for each modality).