Paragangliomas are rare tumors that form near the carotid artery, along nerve pathways in the head and neck, and in other parts of the body. They form outside the adrenal gland. Some paragangliomas make extra catecholamines called adrenaline and noradrenaline. The release of these extra catecholamines into the blood may cause signs or symptoms of disease.

Signs & Symptoms

Signs and symptoms of pheochromocytoma and paraganglioma include high blood pressure and headache. Some tumors do not make extra adrenaline or noradrenaline and do not cause signs and symptoms. These tumors are sometimes found when a lump forms in the neck or when a test or procedure is done for another reason. Signs and symptoms of pheochromocytoma and paraganglioma occur when too much adrenaline or noradrenaline is released into the blood. These and other signs and symptoms may be caused by pheochromocytoma and paraganglioma or by other conditions. Check with your doctor if you have any of the following:

  • High blood pressure
  • Headache
  • Heavy sweating for no known reason
  • A strong, fast, or irregular heartbeat
  • Being shaky
  • Being extremely pale.

The most common sign is high blood pressure. It may be hard to control. Very high blood pressure can cause serious health problems such as irregular heartbeat, heart attack, stroke, or death.

Treatment

Your OHC doctor will help you determine the best care plan for you.

Localized Pheochromocytoma and Paraganglioma

Treatment of localized benign pheochromocytoma or paraganglioma is usually surgery to completely remove the tumor. If the tumor is in the adrenal gland, the entire adrenal gland is removed.

Inherited Pheochromocytoma:

  • In patients with inherited pheochromocytoma linked to multiple endocrine neoplasia (MEN2) or von Hippel-Lindau (VHL) syndrome, tumors often form in both adrenal glands. The tumors are usually benign.
  • Treatment for inherited pheochromocytoma that forms in one adrenal gland is surgery to completely remove the gland. This surgery may help patients avoid lifelong steroid hormone replacement therapy and acute adrenal insufficiency.
  • Treatment for inherited pheochromocytoma that forms in both adrenal glands or later forms in the remaining adrenal gland may be surgery to remove the tumor and as little normal tissue in the adrenal cortex as possible. This surgery may help patients avoid life-long hormone replacement therapy and health problems due to the loss of hormones made by the adrenal gland.

Regional Pheochromocytoma and Paraganglioma

Treatment of pheochromocytoma or paraganglioma that has spread to nearby organs or lymph nodes is surgery to completely remove the tumor. Nearby organs that the cancer has spread to, such as the kidney, liver, part of a major blood vessel, and lymph nodes, may also be removed.

Metastatic Pheochromocytoma and Paraganglioma

Treatment of metastatic pheochromocytoma or paraganglioma may include the following:

  • Surgery to completely remove the cancer, including tumors that have spread to distant parts of the body
  • Palliative therapy, to relieve symptoms and improve the quality of life, including:
    • Surgery to remove as much cancer as possible
    • Combination chemotherapy
    • Radiation therapy with 131I-MIBG
    • External radiation therapy to areas (such as bone) where cancer has spread and cannot be removed by surgery
    • Embolization (treatment to block an artery that supplies blood to a tumor)
    • Ablation therapy using radiofrequency ablation or cryoablation for tumors in the liver or bone
  • A clinical trial of targeted therapy with a tyrosine kinase inhibitor
  • A clinical trial of internal radiation therapy using a new radioactive substance.

Recurrent Pheochromocytoma and Paraganglioma

Treatment of recurrent pheochromocytoma or paraganglioma may include the following:

  • Surgery to completely remove the cancer.
  • When surgery to remove the cancer is not possible, palliative therapy to relieve symptoms and improve the quality of life, including:
    • Combination chemotherapy
    • Targeted therapy
    • Radiation therapy using 131I-MIBG
    • External radiation therapy to areas (such as bone) where cancer has spread and cannot be removed by surgery
    • Ablation therapy using radiofrequency ablation or cryoablation