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Adrenal gland NETs

OVERVIEW

Adrenal gland NETs

Pheochromocytoma and paraganglioma (also called Pheo/Para) are rare NETs that occur either inside or outside of the adrenal glands. These tumors affect approximately 1 in every 3,000 people.

WHERE ARE THE ADRENAL GLANDS?

There are two adrenal glands, located on the top of each kidney in the back of the upper abdomen. Adrenal glands make hormones that control blood pressure and blood sugar
to help the body respond to stress.

What are pheochromocytomas and paragangliomas?

Pheochromocytomas: occur in nerve tissue inside the adrenal glands and near certain blood vessels and nerves.

Paragangliomas: occur outside the adrenal glands. Pheo/para produce excess amounts of adrenaline and related hormones, which are the “fight-or-flight” hormones usually released in response to stress.

Pheo and para symptoms

When pheo and para tumors release too much adrenaline or noradrenaline, it may cause:

  • High blood pressure
  • Headache
  • Heavy sweating without any known reason
  • A strong, fast, or irregular heartbeat
  • Shakiness
  • Extreme paleness

The signs and symptoms of pheo and para may occur after:

  • Strenuous physical activity
  • Physical injury
  • Emotional stress
  • Childbirth
  • Undergoing anesthesia
  • Surgery (which may include surgery to remove the tumor)
  • Eating foods and drinks that are high in tyramine (such as red wine, chocolate, and some meats and cheeses)

Risk factors for pheochromocytoma and paraganglioma

Sometimes specific syndromes or gene changes passed down in families can increase the risk of pheochromocytoma or paraganglioma. These risk factors include:

  • Multiple endocrine neoplasia 2 syndrome types A and B (MEN2A and MEN2B)
  • Von Hippel-Lindau (VHL) disease
  • Neurofibromatosis type 1 (NF1)
  • Hereditary paraganglioma syndrome (succinate dehydrogenase mutations)
  • Carney-Stratakis dyad
  • Carney triad

Having someone in your family with one of these risk factors does not mean you will develop this type of cancer; however, it increases the likelihood.

NET SITEs

Learn more about these NET sites.

NETs can occur throughout the body, but most commonly form in the GI tract, pancreas, lungs and adrenal glands.

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