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What is pheochromocytoma & paraganglioma?
Pheochromocytoma and paraganglioma are rare tumors that grow from the same special nerve-related cells that make adrenaline and similar 'fight-or-flight' hormones. When the tumor starts in the inner part of an adrenal gland (the gland sitting on top of each kidney), it is called a pheochromocytoma. When it starts in similar cells found elsewhere along the spine, in the chest, abdomen, or pelvis, it is called a paraganglioma. Many of these tumors are 'functioning,' meaning they pour extra adrenaline-type hormones into the blood. That can cause spells of pounding heartbeat, high blood pressure, sweating, headache, and anxiety. Because of these hormone surges, the care team takes special steps with medicines before any procedure to keep blood pressure safe. A good share of these tumors run in families or are linked to inherited gene changes, so genetic counseling is often offered. Most are slow-growing and curable with surgery, but some can spread, and those cases are managed by an experienced team using surgery, special targeted radiation, and medicine.
The main types
Doctors group pheochromocytoma & paraganglioma by where it starts and how it behaves:
| Type | What it means, simply |
|---|---|
| Pheochromocytoma | A tumor in the inner part (medulla) of an adrenal gland that often makes extra adrenaline-like hormones. |
| Paraganglioma | A tumor of the same cell type found outside the adrenal gland — along the spine, in the head and neck, chest, or belly. |
| Functioning tumor | A tumor that releases extra hormones, causing spells of high blood pressure, racing heart, sweating, and headache. |
| Hereditary tumor | A tumor linked to an inherited gene change (such as SDHB or others); these can run in families, so genetic testing is often offered. |
Staging, in plain terms
Unlike many cancers, these tumors are not grouped mainly by a number stage. Doctors describe them as localized (still in one place), regional (spread to nearby lymph nodes), or metastatic (spread to distant sites such as bone, liver, or lung). Because any of these tumors can behave aggressively, the team looks at the tumor's location and size, blood and urine hormone levels, special scans that light up these tumors, and inherited gene results to judge risk and plan treatment. There is no truly 'benign' label — all are watched carefully over time.
| Localized vs. metastatic (with genetic and hormone evaluation) | What it generally means |
|---|---|
| Localized | The tumor is in one place and has not spread — usually curable with surgery after careful blood-pressure preparation. |
| Regional | The tumor has spread to nearby lymph nodes or tissue but not to distant organs. |
| Metastatic | The tumor has spread to distant sites such as bone, liver, or lung — managed with surgery, special targeted radiation, and medicine to control growth and hormones. |
| Hereditary / multifocal | More than one tumor, or a tumor tied to an inherited gene change — followed closely with imaging and family genetic counseling. |
The standard of care
Pheochromocytoma & Paraganglioma is almost always treated by a team that may include a surgeon, a medical oncologist, and a radiation oncologist, combining therapies for the best result. The usual building blocks are:
Blood-pressure preparation
Before any surgery or procedure, medicines (alpha blockers) are given for one to several weeks to block the hormone surges and keep blood pressure safe.
Surgery
Carefully removing the tumor by an experienced surgeon is the main treatment and can cure localized disease.
Targeted radiation therapy (theranostics)
For tumors that have spread and take up special tracers, radioactive medicines (MIBG or peptide-receptor therapy) deliver radiation directly to tumor cells throughout the body.
External radiation and supportive care
Focused external radiation can control or relieve specific spots, especially in bone, and medicines help manage hormone symptoms.
How radiation treatment works
Radiation treats these tumors in two ways. The first is from the outside, using focused high-energy x-rays aimed precisely at a tumor to damage its DNA so the cells can no longer grow — useful for controlling or relieving specific spots such as in bone or the spine. The second, and often more important here, is radiation delivered from the inside. Because pheochromocytomas and paragangliomas absorb certain molecules, doctors can attach a radioactive tag to one of those molecules and give it through a vein. It then travels through the bloodstream and concentrates inside tumor cells wherever they are, irradiating them from within while largely sparing healthy tissue. This is called radiopharmaceutical therapy or theranostics. Treatments are planned by a specialized team, and because these tumors can release hormones, blood pressure is managed carefully throughout. Side effects are usually manageable and depend on the approach used.
The main ways radiation is delivered for pheochromocytoma & paraganglioma:
Radiopharmaceutical therapy (I-131 MIBG)
A radioactive form of a molecule these tumors absorb is given through a vein; it travels to tumor cells anywhere in the body and delivers radiation from the inside.
Peptide receptor radionuclide therapy (PRRT)
For tumors with the right surface markers, a radioactive medicine binds to the tumor cells and irradiates them directly, sparing most normal tissue.
Stereotactic body radiation (SBRT)
Highly focused external radiation in a few sessions can control a limited number of metastatic spots, such as in bone or the spine, while protecting nearby organs.
Palliative external radiation
Precise radiation aimed at painful deposits relieves symptoms and improves comfort.
Latest studies shaping care
Care keeps improving — often toward getting the same excellent results with less burden on patients. A few developments:
High-specific-activity MIBG for spread disease: A purified radioactive MIBG therapy was shown to control tumors and reduce blood-pressure medication needs in people with metastatic pheochromocytoma and paraganglioma, leading to its approval.[1]
Pivotal iobenguane I-131 trial; FDA label
Peptide receptor radionuclide therapy (PRRT): Radioactive somatostatin-targeting therapy, already used for other neuroendocrine tumors, shows promising control of metastatic paraganglioma in tumors with the right receptors.[2]
Multi-center PRRT series; ESMO updates
Genetic testing guides care: Because many of these tumors carry inherited gene changes, guidelines now recommend genetic counseling and testing for nearly all patients to guide screening and treatment.[3]
Endocrine Society and international consensus guidelines
Common questions
Why do I need medicine before surgery? These tumors can release surges of adrenaline-like hormones that spike blood pressure during a procedure. Taking blood-pressure medicines (alpha blockers) for a few weeks beforehand makes surgery much safer. Your team will guide the timing carefully.
Should my family be tested? Often, yes. A meaningful share of these tumors are linked to inherited gene changes. Genetic counseling and testing can show whether relatives should be screened and can guide your own follow-up. Your team can arrange this.
What if the tumor has spread? Spread disease is managed by an experienced team. Options include special radioactive medicines that target tumor cells throughout the body, focused external radiation for specific spots, surgery when helpful, and medicines to control hormones and growth.
References
Numbered sources for the studies cited above. Links open the primary publication on PubMed or the publisher’s site.