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What is pheochromocytoma?

Pheochromocytoma is a rare type of tumor that occurs in or near the adrenal glands. This tumor produces too many of the hormones called catecholamines, often referred to as “flight and fright hormones.”

It occurs in less than 0.2% of people with high blood pressure. The annual incidence of this tumor is 0.8 per 100,000 person-years. The highest incidence is in the age group 30 to 40, although it can occur at any age in both males and females.

Approximately 95 % of catecholamine-secreting tumors are in the abdomen, 85 to 90 percent of which are intraadrenal (pheochromocytoma), and 5 to 10 percent are multiple. Approximately 10 to 15 percent of catecholamine-secreting tumors are extra-adrenal and are referred to as catecholamine-secreting paragangliomas.

There are mainly two causes of pheochromocytoma:

  1. Most cases are sporadic (occur spontaneously)
  2. Hereditary or part of a syndrome that runs in families.

Symptoms of pheochromocytoma are:

  1. Classic triad of symptoms: Episodes of headache, rapid heartbeat, sweating
  2. Shortness of breath
  3. Anxiety
  4. Sometimes, it causes a feeling of impending doom.

Signs of pheochromocytoma are:

  1. High blood pressure is difficult to control with medications or sometimes gets worse with medications like beta-blockers, or they may have extremely high blood pressure during surgery or after a medical procedure.
  2. Heart failure.

Diagnosing primary aldosteronism requires a high degree of clinical suspicion to diagnose and successfully treat this condition. Dr Thomas will do a comprehensive medical evaluation by eliciting all the different symptoms and signs of the condition, getting a relevant personal and family history, and doing a thorough physical examination. She will order the relevant blood and urine tests to help diagnose this condition. She will arrange for you to have the appropriate radiology imaging – either a CT scan or MRI of the adrenal gland for localization of the adrenal gland tumor. She will also recommend any further imaging studies if needed.

She will also recommend genetic testing as 40% of this disorder is part of an inherited disorder.

The overall treatment goal in patients with pheochromocytoma is to prevent the adverse outcomes associated with high blood pressure and the impact of high catecholamines on the blood vessels, brain, heart, and kidneys.

Dr Thomas will review all the results of the blood tests, urine studies, and relevant imaging studies (CT scan/MRI adrenal glands). She will educate you on the condition, explain all the results of the tests, discuss your treatment options, and formulate an individualized treatment plan for you.

For an adrenal gland tumor producing excess catecholamines, Dr Thomas will refer you to an experienced endocrine glands surgeon for surgery to remove the adrenal gland that is producing excess pheochromocytoma. She will help prepare you for surgery by advising you on dietary and fluid intake and optimizing your blood pressure regimen. These are important considerations before you have your surgery.

Surgical removal of the pheochromocytoma does not always lead to a long-term cure for high blood pressure. Recurrence of the tumor can occur even in benign tumors, more likely in familial pheochromocytoma, right adrenal tumors, and extra-adrenal tumors.

During your follow-up with Dr Thomas, she will do a comprehensive medical evaluation and arrange for you to have relevant blood tests and urine studies to monitor for any recurrence of the tumor. She will monitor your blood pressure, adjust your blood pressure medications, and treat any other cardiovascular risk factors, which are important to prevent heart disease, strokes, and kidney disease.

At a Glance

Dr. Reena Thomas, MD

  • Dual American board-certified endocrinologist
  • Author of numerous academic and clinic research
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