management

Last reviewed 01/2018

Initial pharmacological treatment permits optimal surgical interventions.

  • medical
    • objective - to control the adverse effects of circulating catecholamines until definitive surgery
      • alpha blockers (phenoxybenzamine, metyrosine, prazosin) to control hypertension. Once alpha blockers are used, if tachycardia is present
      • beta blockers (oral propranolol) - prevents catecholamine-induced arrhythmias
      • phentolamine or nitroprusside - hypertensive crises during and before surgery
      • hypotension is a post-op complication - prevented with adequate volume expansion preoperatively.

The tumour is localised using the following imaging methods:

  • CT scan - which can detect tumours greater than 1 cm in diameter
  • 131 I-meta-iodobenzylguanidine (MIBG) scintigraphy:
    • more sensitive than CT
    • MIBG is specifically concentrated in adrenergic cells and is useful for assessing metastases

Surgery is curative in 75% of cases

  • for adrenal phaeochromocytomas
    • adrenalectomy following pre-op stabilization
      • laparoscopic adrenolectomy
      • transabdominal approach
  • excessive handling of the tumour peroperatively should be avoided as this may release large amounts of catecholamine into the circulation resulting in severe, acute hypertension
  • vasodilators such as sodium nitroprusside, and intra-venous alpha- and beta-blockers should be available during surgery in the event of a hypertensive crisis
  • operative mortality is at least 3%.

When surgery is not possible, consider treatment with:

  • phenoxybenzamine and propranolol
  • alpha-methyl-p-tyrosine - inhibits the hydroxylation of tyrosine to dopa and so, reduces synthesis of adrenaline and noradrenaline

combination chemotherapy if malignant:

  • cyclophosphamide
  • vincristine
  • dacarbazine