Watch: the CureRays® explainer series
Prefer to read? The full guide below is complete on its own, with tables you can revisit any time.
What is paraganglioma?
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.
The main types
Doctors group paraganglioma by where it starts and how it behaves:
| Type | What it means, simply |
|---|---|
| Head and neck paraganglioma | Includes carotid body and other skull-base/neck tumors; usually does not make hormones and tends to grow slowly, causing a neck mass or nerve symptoms. |
| Sympathetic (abdominal/pelvic/chest) paraganglioma | Often makes adrenaline-like hormones, causing blood-pressure spells; managed much like an adrenal pheochromocytoma, with careful preparation before surgery. |
| Hereditary paraganglioma | Linked to inherited gene changes (SDHB, SDHD, and others). These can be multiple, can recur, and — with SDHB especially — carry a higher risk of spreading, so lifelong screening is advised. |
Staging, in plain terms
Paraganglioma uses a TNM system shared with pheochromocytoma that considers tumor size and location, lymph-node involvement, and distant spread. Because behavior is hard to predict from appearance alone, doctors also weigh genetic findings (especially SDHB) and the tumor's location when planning treatment and follow-up.
| Pheochromocytoma/paraganglioma TNM staging | What it generally means |
|---|---|
| Localized | Tumor confined to where it started, with no spread. Usually treated and often cured with surgery. |
| Regional | Spread to nearby lymph nodes or tissues. Surgery is still central, sometimes combined with other treatments. |
| Metastatic | Spread to distant sites such as bone, liver, or lungs. Managed long-term with targeted radionuclide therapy, surgery, and other treatments, often over many years. |
The standard of care
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:
Surgery (the main treatment)
Removing the tumor is the cornerstone and is often curative for localized disease. Hormone-producing tumors need careful medical preparation first to control blood pressure safely during surgery.
Blood-pressure preparation
For tumors that make adrenaline-like hormones, medicines (alpha-blockers, then beta-blockers) are started before surgery to prevent dangerous blood-pressure swings.
Targeted radionuclide therapy (PRRT / MIBG)
For tumors that have spread, treatments like Lutetium-177 DOTATATE or I-131 MIBG deliver radiation directly to tumor cells from the inside, controlling disease and easing symptoms.
Genetic counseling and family screening
Because many paragangliomas are inherited, genetic testing is recommended, and at-risk relatives may be screened so any tumors are found early.
How radiation treatment works
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 main ways radiation is delivered for paraganglioma:
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.
Latest studies shaping care
Care keeps improving — often toward getting the same excellent results with less burden on patients. A few developments:
Lutetium-177 DOTATATE for spread disease: Targeted radionuclide therapy that homes to paraganglioma cells has become a leading option for tumors that have spread, controlling disease and improving symptoms in patients who were once hard to treat.[1]
Neuroendocrine radionuclide-therapy studies (2021–2025)
Genetics reshapes screening and care: Recognition that a large share of paragangliomas are hereditary — particularly SDHB, SDHD, and related genes — has made genetic testing and lifelong surveillance standard, helping catch new or recurrent tumors early in patients and relatives.[2]
Endocrine genetics and surveillance guidelines (2020–2025)
Radiosurgery for head-and-neck tumors: Series of carotid-body and skull-base paragangliomas show that stereotactic radiosurgery can control tumor growth with low risk to nearby nerves, offering an alternative to surgery in delicate locations.[3]
Head-and-neck and radiosurgery outcome studies (2020–2024)
Common questions
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.
References
Numbered sources for the studies cited above. Links open the primary publication on PubMed or the publisher’s site.
- Neuroendocrine radionuclide-therapy studies (2021–2025) (no indexed identifier — see your care team) ↩
- Endocrine genetics and surveillance guidelines (2020–2025) (no indexed identifier — see your care team) ↩
- Head-and-neck and radiosurgery outcome studies (2020–2024) (no indexed identifier — see your care team) ↩