Pheochromocytoma and Paraganglioma Treatment Options

Despite the low incidence of pheochromocytoma, it is important to screen, detect, and treat the condition early since it can be cured in 90% of cases. However, if left untreated, the tumor is likely to be fatal due to catecholamine-induced malignant hypertension, heart failure, myocardial infarction, stroke, ventricular arrhythmias, or metastatic disease.

The treatment of first choice for pheochromocytoma and paraganglioma is surgical resection, with laparoscopic surgery being the preferred technique for tumors located in the adrenal gland. For unresectable tumors, alternative treatment approaches should be considered.

Several clinical trials assessing the safety and efficacy of chemotherapy in treating pheochromocytoma and paraganglioma are ongoing. For information on current clinical trials on treatment of pheochromocytoma and paraganglioma, please see Research.

Preoperative Management

All patients with pheochromocytoma and paraganglioma undergoing surgery should receive preoperative medical management to minimize operative and postoperative complications. The main goal of preoperative management is to normalize blood pressure, heart rate, and function of other organs; restore volume depletion; and protect the patient from the effects of high levels of circulating catecholamines during surgery, which may cause hypertensive crises and arrhythmias. Agents used for preoperative management include α-adrenergic antagonists, β-adrenergic antagonists, calcium channel blockers, and catecholamine synthesis inhibitors.