PHEOCHROMOCYTOMAS AND PARAGANGLIOMAS

In the medical world, we tend to use lots of words that take some time to roll off the tongue. This week’s blog post deals with two of those words: Pheochromocytomas and Paragangliomas.

First of all, let’s figure out how to say these words.

Pheochromocytomas breaks down like this: pheo·chro·mo·cy·to·ma.

Paragangliomas breaks down like this: para·gan·gli·o·ma·ta.

Got it?

Now what are pheochromocytomas and paragangliomas?

Pheochromocytomas are tumors that originate inside the medulla of the adrenal glands. Paragangliomas are tumors that originate in the organs of the autonomic nervous system outside of the adrenal glands, meaning they can be located in the head and neck, or in the thoracic, abdominal, and pelvic cavities. MEN2A patients usually have benign pheochromocytoomas and not paragangliomas..

Because of their tissue of origin, pheochromocytomas and paragangliomas may produce hormones called catecholamines (adrenaline and norepinephrine). When these hormones are produced excessively, high blood pressure and heart palpitations may result, predisposing the patient to heart attacks, strokes, heart failure, and abnormal heart rhythms. Additionally, the excessive production of catecholamines may induce headaches, tremors, palor and excessive sweating.

Treatment of pheochromocytoma in MEN2A patients is surgery to remove the affected adrenal gland or affected portion of the gland. Prior to surgery, you must undergo alpha blockade in order to make anesthesia safe for you.

If surgery is not possible, the treatment of pheochromocytomas and paragangliomas includes the use of medications that prevent complications due to excessive hormone secretion, as well as medications that directly attack the tumor.

Medications that control hormone abnormalities include alpha-blockers such as phenoxynenzamine and terazosin, beta-blockers like metoprolol, atenolol, and propranolol, and medications that decrease the synthesis of catecholamines, e.g. metyrosine. These medications can be used to control hypertension and to prevent cardiovascular disease.