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Pheochromocytoma and Paraganglioma Treatment (PDQ®)

Treatment Option Overview

Definitive treatment for localized and regional pheochromocytoma, including localized disease recurrence, consists of alpha- and beta-adrenergic blockade followed by surgery. For patients with unresectable or metastatic disease, treatment may include a combination of the following:

  • Catecholamine blockade.
  • Surgery.
  • Chemotherapy.
  • Radiofrequency ablation.
  • Cryoablation.
  • Radiation therapy.

Only limited data are available from phase II clinical trials to guide the management of patients diagnosed with pheochromocytoma or paraganglioma. There are no phase III trials. Everything is based on case series, and the impact of survival is not known.

Treatment for patients with localized, regional, metastatic, or recurrent pheochromocytoma is summarized in Table 2.

Table 2. Treatment Options for Patients With Pheochromocytoma
PheochromocytomaStandard Treatment Options
Localized PheochromocytomaSurgery
Regional PheochromocytomaSurgery
Metastatic PheochromocytomaSurgery
Palliative therapy
Recurrent PheochromocytomaSurgery
Palliative therapy

Preoperative Medical Preparation

Surgery is the mainstay of treatment for most patients; however, preoperative medical preparation is critical. Alpha-adrenergic blockade should be initiated at the time of diagnosis and maximized preoperatively to prevent potentially life-threatening cardiovascular complications, which can occur as a result of excess catecholamine secretion during surgery. Complications may include the following:

  • Hypertensive crisis.
  • Arrhythmia.
  • Myocardial infarction.
  • Pulmonary edema.

Phenoxybenzamine (a nonselective alpha-antagonist) is the usual drug of choice; prazosin, terazosin, and doxazosin (selective alpha-1-antagonists) are alternative choices.[1,2] Prazosin, terazosin, and doxazosin are shorter acting than phenoxybenzamine, and therefore, the duration of postoperative hypotension is theoretically less than with phenoxybenzamine; however, there is less overall experience with selective alpha-1-antagonists than with phenoxybenzamine.

A preoperative treatment period of 1 to 3 weeks is usually sufficient; resolution of spells and a target low normal blood pressure for age indicate that alpha-adrenergic blockade is adequate. During alpha-adrenergic blockade, liberal salt and fluid intake should be encouraged because volume loading reduces excessive orthostatic hypotension both preoperatively and postoperatively. If tachycardia develops or if blood pressure control is not optimal with alpha-adrenergic blockade, a beta-adrenergic blocker (e.g., metoprolol or propranolol) can be added but only after alpha-blockade. Beta-adrenergic blockade must never be initiated before alpha-adrenergic blockade; doing so blocks beta-adrenergic receptor-mediated vasodilation and results in unopposed alpha-adrenergic receptor-mediated vasoconstriction, which can lead to a life-threatening crisis.

References

  1. Cubeddu LX, Zarate NA, Rosales CB, et al.: Prazosin and propranolol in preoperative management of pheochromocytoma. Clin Pharmacol Ther 32 (2): 156-60, 1982. [PUBMED Abstract]
  2. Prys-Roberts C, Farndon JR: Efficacy and safety of doxazosin for perioperative management of patients with pheochromocytoma. World J Surg 26 (8): 1037-42, 2002. [PUBMED Abstract]
  • Updated: October 24, 2013