The American Association of Endocrine Surgeons, Patient Education Site

Paraganglioma (adrenaline-producing tumor outside the adrenal gland)

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What if I have recurrent or metastatic disease?

Recurrent Disease (i.e. disease that comes back)

Recurrent tumor at the original site is best managed with another operation. Reoperations may require removal of the kidney, other surrounding organs and/or parts of major blood vessels in order to completely remove the disease. Tumors that recur in the adrenal gland are treated by total or partial adrenalectomyPartial adrenalectomy - an operation to remove the part of an adrenal gland that has the tumor. Also called a cortical-sparing adrenalectomy.. MetastasesMetastases - cancer that has spread from another part of the body to other organs (such as painful bone metastases) might be removed or treated with radiofrequency ablation or surgery.

Metastatic Disease (i.e. disease that has spread to other parts of the body)

Unfortunately, once metastases develop, cure is no longer possible. At this point, the goals of treatment are palliation (i.e. reducing hypertension, "spells", and pain) as well as prolonging life. Partial surgical removal (termed palliative debulking) is rarely, but sometimes an option. Pharmacologic blockade is maintained with alpha and beta blockers. Radiofrequency ablation can be considered in patients with a small number of metastases in the liver or for those with painful bone metastases. Systemic chemotherapy is not curative, but does have a role in patients who have disease that has not responded to other treatments. Treatment with high-dose 131I MIBG has shown some benefit for treating widespread metastases, but not without serious side effects.10 More recently, the use of a new medication sunitinib (a tyrosine kinase inhibitor) has demonstrated some success in patients with metastatic disease. However, these drugs have not been approved by the US Federal Drug Administration for treatment of patients with malignant pheochromocytomaPheochromocytoma - a disease where too much adrenaline is being made by an adrenal tumor/paragangliomaParaganglioma - a disease where too much adrenaline is being made by a tumor outside the adrenal glands. Patients with disease that has not responded to traditional therapy should consider entering a clinical trial. Available clinical trials are best investigated though the Clinical Trials home page of the National Cancer Institute at www.cancer.gov/clinicaltrials.

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