Paraganglioma

Diagnosis

Paraganglioma diagnosis often begins with blood and urine tests that look for signs of excess hormones made by the tumor. These tests can identify elevated catecholamines or proteins such as chromogranin A, which may suggest a paraganglioma. Depending on symptoms and risk factors, additional imaging and genetic tests may be recommended.

Blood and urine tests measure hormone levels to detect extra catecholamines made by a tumor or other markers related to paraganglioma. If results indicate a possible tumor, imaging tests may follow to determine the size, location and possible spread of the growth. These imaging tests can also help guide treatment decisions.

Imaging tests often used for paraganglioma include:

• Magnetic resonance imaging, which uses radio waves and magnets to create detailed images

• Computerized tomography scans that combine multiple X-ray images from different angles

• MIBG scans that track a radioactive tracer absorbed by paragangliomas

• PET scans that detect a tracer taken up by active tumor cells

Genetic testing may be advised because some inherited DNA changes increase the likelihood of developing paragangliomas. Test results may help predict whether a tumor could return after treatment. Family members may also be offered testing to identify those who carry similar genetic risks. A genetic counselor can guide discussions about testing and help families understand their options.

Treatment

Most paragangliomas are treated with surgery to remove the tumor. When a tumor produces hormones, medicines are often used first to block or reduce these effects. If the tumor cannot be fully removed or has spread to other areas, additional treatments may be considered based on the tumor’s location, whether it is cancerous and the presence of hormone-related symptoms.

Treatments that control excess hormones may be needed if the tumor produces catecholamines. These treatments help manage high blood pressure and other symptoms. Alpha blockers, beta blockers and calcium channel blockers may be used. Increasing sodium intake and staying well hydrated may also be recommended. Symptoms and blood pressure must be controlled before other treatments begin to avoid complications triggered by hormone surges during procedures.

Surgery may be performed to remove the tumor. Even when full removal is not possible, partial removal can help reduce symptoms. The type of surgeon involved depends on the tumor’s location. Head and neck tumors may be treated by specialists in that region, while tumors near the brain or nerves may require neurosurgeons. Tumors of hormone-producing glands may involve endocrine surgeons, and tumors affecting blood vessels may require vascular surgeons. In some cases, multiple specialists work together.

Radiation therapy may be used when surgery cannot remove the entire tumor or when the tumor has spread. It can also help ease pain from tumors in other parts of the body. Stereotactic radiosurgery may be used for tumors in the head and neck area. This method directs many small beams of radiation at the tumor to deliver a strong dose where the beams meet.

Ablation therapy may be recommended when a paraganglioma spreads. This treatment uses heat or cold to destroy tumor cells. Radiofrequency ablation heats tumor cells, while cryoablation freezes them to stop growth.

Chemotherapy may be used when a paraganglioma has spread to distant areas. Strong medicines are given to shrink the tumors. If the tumor makes hormones, medicines to control hormone levels are given first to prevent complications.

Targeted therapy uses medicines that block specific chemicals inside tumor cells. These treatments may be recommended when surgery is not possible or when the paraganglioma has spread.

Peptide receptor radionuclide therapy delivers radiation directly to tumor cells using a medicine infused through a vein. This medicine attaches to paraganglioma cells and releases radiation over several days. Lutetium Lu 177 dotatate may be used when tumors cannot be removed or have spread.

Clinical trials may offer access to new or developing treatments. Your healthcare professional can help determine whether a trial is appropriate and discuss potential benefits and risks.

Watchful waiting may be recommended when a paraganglioma grows slowly and does not cause symptoms. Regular monitoring allows healthcare professionals to track changes and decide when treatment may become necessary.