Adrenal gland NETs

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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.

GI Tract

Pancreas

Lung

Adrenal

Liver Mets

Tumor Sites Overview

To Learn More on This Topic...

Read NETRF’s Blog

Understanding Your Neuroendocrine Tumor Diagnosis
Inherited Risk Factors for NETs

Listen to Our Netwise Podcasts

Special Episode: Small Bowel NETs
Episode 10: Pancreatic NETs
Episode 14: Pheos and Paras—NETs Known as “the Great Mimic”
Episode 17: Focus on Liver NETs
Episode 32: Pheos & Paras

Get Insights from the Experts

PNETs, by Xavier Keutgen, MD, FACS, and Chih-Yi (Andy) Liao, MD
SiNETs, by Chandrikha Chandrasekharan, MBBS
Lung NETs, by Suki Padda, MD
Pheo/Para and Genetics, by Lauren Fishbein, MD, PhD
GI Troubles, Diarrhea and Diet, by David Richards, MD
Lung Neuroendocrine Tumors, by Suki Padda, MD
Keeping Your Liver Healthy, by Helen Te, MD
Know Your Lung NET, by Robert Ramirez, DO
Carcinoid Heart Disease, by Heidi Connolly, MD
NETs Risk Factors, by Lauren Fishbein, MD, PhD

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Adrenal gland NETs

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