Paraganglioma, from the CureRays guide library.

Paraganglioma is a rare tumor of nerve-related hormone tissue found outside the adrenal gland — often slow-growing and frequently inherited — treated mainly with surgery, with a special 'radiation from within' therapy (Lutetium-177 DOTATATE) for tumors that have spread.

What it is.

Paraganglioma is a rare tumor that grows from clusters of specialized nerve-related cells called paraganglia, which sit along nerves and blood vessels from the base of the skull and neck down through the chest, abdomen, and pelvis. It is closely related to pheochromocytoma, which is the same kind of tumor when it occurs inside the adrenal gland — paragangliomas are simply found outside it. Some paragangliomas, especially those in the abdomen, make adrenaline-like hormones that can cause spells of high blood pressure, pounding heartbeat, sweating, and headaches; others, particularly in the head and neck, make little or no hormone and instead cause a lump or pressure on nearby nerves. Paragangliomas are notable for being one of the most strongly inherited of all tumors — a large share are linked to gene changes in the SDHB, SDHD, and related genes — so genetic counseling and testing are an important part of care for patients and their families. Most paragangliomas are slow-growing, but a minority can spread, and the SDHB gene change in particular signals a higher risk of that.

How radiation treats it.

Radiation damages the DNA inside cancer cells so they can no longer divide and survive, while normal cells are better at repairing themselves. Paraganglioma can be treated with radiation in two very different ways. From the outside, focused external-beam radiation or stereotactic radiosurgery can control a tumor sitting among delicate nerves and blood vessels in the head and neck without an operation. From the inside, targeted radionuclide therapy (such as Lutetium-177 DOTATATE) uses a molecule that seeks out paraganglioma cells and carries a radioactive payload straight to them — delivering radiation to tumors throughout the body while sparing most healthy tissue. With this internal therapy you may need brief precautions for a short time, which your team will explain; external radiation leaves no radioactivity in your body.

The ways we can treat it.

Surgery. Carefully removing the tumor — often by surgeons experienced with its rich blood supply and nerve neighbors — is the primary, potentially curative treatment for localized disease.

External-beam radiation / radiosurgery. For head-and-neck paragangliomas near critical nerves and blood vessels, focused external radiation or stereotactic radiosurgery can control the tumor while avoiding the risks of surgery, making it an attractive alternative there.

Targeted radionuclide therapy (PRRT). Lutetium-177 DOTATATE is a 'radiation from within' treatment: a molecule that homes to paraganglioma cells carries a radioactive payload directly to them, delivering radiation to tumors throughout the body while largely sparing healthy tissue.

Questions we hear often.

Should my family be tested too? Possibly. Paraganglioma is one of the most strongly inherited tumors, often linked to gene changes such as SDHB or SDHD. Genetic counseling and testing are usually recommended, and if an inherited change is found, relatives can be screened so any tumors are caught early.

What is 'radiation from within' for paraganglioma? For tumors that have spread, a treatment called targeted radionuclide therapy (for example Lutetium-177 DOTATATE) uses a molecule that seeks out paraganglioma cells and delivers a radioactive payload directly to them. This treats tumors throughout the body while sparing most healthy tissue.

Why do I need medicines before surgery? If your tumor makes adrenaline-like hormones, your team will start blood-pressure medicines (usually an alpha-blocker, then a beta-blocker) for a couple of weeks before surgery. This prevents dangerous blood-pressure spikes when the tumor is handled during the operation.

This guide is informational only. It is not medical advice — please confirm anything here with your care team.