Metastatic ("malignant") pheochromocytoma and paraganglioma: is pheochromocytoma cancer?

Pheochromocytomas and paragangliomas are neuroendocrine tumors originating in the paraganglia. Pheochromocytomas originate in the adrenal medulla, and paragangliomas originate in the paraganglia outside the adrenal glands. Most of these tumors secrete excessive amounts of catecholamines (adrenaline-type hormones) that put patients at risk of devastating complications (heart, blood vessels, abdomen, brain, kidneys, etc.).

These tumors used to be divided into "benign" and "malignant". That is no longer done, because no test, scan or microscope finding can reliably tell which tumors will spread. Today every pheochromocytoma and paraganglioma is considered to have some potential to spread (metastasize), and the term used is "metastatic" pheochromocytoma when the tumor has spread to other parts of the body. Most pheochromocytomas never spread. The risk is higher for paragangliomas and for people who carry a change in the SDHB gene, which carries the highest metastatic risk of all the hereditary genes. This is why genetic testing should be offered to every patient (about 30–40% of these tumors are hereditary; see our pheochromocytoma genetics page), and why follow-up with a metanephrine test every year is lifelong: spread or recurrence can appear many years after the first operation.

Surgery to remove the tumor is the main treatment. For most pheochromocytomas our preferred operation is the Mini Back Scope Adrenalectomy (MBSA), performed with a scope through three small incisions in the lower part of the back. Since the adrenal glands are in the very back part of your abdomen, behind all the other organs, getting to them through the back makes sense and has several advantages. Very large tumors, and tumors that are known or suspected to have invaded surrounding structures, may need a different approach, including open surgery. Read more about surgery for pheochromocytoma.

Occasionally, metastatic pheochromocytomas and paragangliomas are widespread and curative surgery is no longer possible. Even then, surgery may still have a role in selected patients. Other treatment options include targeted radioactive treatments (such as high-dose MIBG therapy or radioactive DOTATATE therapy, for tumors that take up these substances on the corresponding scan), chemotherapy, and newer targeted drugs, together with medication to control blood pressure. These treatments have side effects that must be weighed carefully, and metastatic disease is best managed by a team with experience in these rare tumors.

Tyrosine kinase inhibitors Several tyrosine kinase inhibitors (TKIs), including axitinib, cabozantinib, lenvatinib, pazopanib, and sunitinib, have been studied in clinical trials for metastatic pheochromocytoma and paraganglioma (www.ClinicalTrials.gov). These agents have in common their capacity to block the activation of the VEGF receptors (VEGFRs), reducing the growth of new blood vessels and of tumor cells. In addition, TKIs can inhibit other tyrosine kinase receptors that are involved in processes such as cancer cell growth, tumor spread, and development of resistance.

Last updated October 2026.