Pheochromocytoma and Paraganglioma, if detected early, can be successfully treated and managed in 90% of cases. The treatment of choice for the condition is surgery. Chemotherapy or radiotherapy as a treatment option is currently being tested in clinical trials.  However, if left untreated, the tumor is likely to be fatal due to high blood pressure, heart failure, stroke, arrhythmias, or metastatic disease (where the cancer spreads to other organs or bones).

Before Surgery
Prior to surgery, all patients should receive medications to minimize any surgery-related complications. The main goal of the administration of these medications is to normalize blood pressure and heart rate and to protect the patient from the effects of high levels of hormones (catecholamines) released during surgery. The medications used include α-adrenergic antagonists, β-adrenergic antagonists, calcium channel blockers, and catecholamine synthesis inhibitors

After Surgery: Follow-Up
Urine and plasma tests should be repeated 14-28 days after surgery to check for any remaining disease. Long-term regular follow-up is recommended for all patients after that. Yearly urine or plasma tests for pheochromocytoma and paraganglioma should be performed for life to detect remaining disease or the return of the disease. Follow-up CT or MRI is not needed if urine and plasma test results are normal.

Cancerous Pheochromocytoma
In 3%-36% of the cases of pheochromocytoma, the disease is cancerous and has spread to other organs.

 

The prognosis for these patients is affected by the location of these tumors. If tumors have spread to the liver and lungs, a patient may have a significantly shorter lifespan than those with tumors in the bones. There are currently no cures for cancerous pheochromocytoma. However, in some cases, the existing treatment options may reduce the tumors and prolong survival.

Current treatment options for cancerous pheochromcytoma include:

 

  • High dose 131I-MIBG
  • Chemotherapy
  • Surgical removal of the tumors
  • Tumor size reduction (surgical debulking)
  • Radiotherapy
  • External-beam irradiations of bone metastases
  • Tumor embolization
  • Radiofrequency ablation
  • Cryoablation (killing of tumor by tissue freezing)