Pheochromocytoma

A pheochromocytoma is a rare tumor that forms in the adrenal glands. These two glands sit above the kidneys and make hormones like adrenaline, which help control heart rate and blood pressure. When a tumor develops, it can cause the body to make too much of these hormones.

Pheochromocytomas are sometimes called pheos. They start in chromaffin cells, a type of cell that makes hormones. These cells are also involved in related tumors called paragangliomas, which can form in other parts of the body.

Many pheochromocytomas grow slowly and are noncancerous, though some can be cancerous. Even when they’re not cancerous, most still need treatment.

Because these tumors are rare and complex, it’s important to get care from an experienced team. At Penn Medicine, specialists from different fields work together to diagnose the tumors and create a treatment plan tailored to each person.

Sometimes pheochromocytoma symptoms are mild or hard to notice. Other times, people experience:

• High blood pressure
• A rapid or pounding heartbeat
• Headaches
• Pale skin
• Sweating
• Tremors or shaking

Less commonly, symptoms include abdominal pain, unintended weight loss, anxiety, nervousness, or panic attacks.

The exact cause is often unknown. In most cases, there are no known lifestyle or environmental risk factors.

Sometimes, pheochromocytomas are linked to inherited genetic changes passed down through families. If a parent carries one of these changes, each child has a 50% chance of inheriting it. But inheriting a gene change doesn’t mean someone will definitely develop a tumor.

Some genetic changes can affect how these tumors behave. They may increase the risk of tumors in both adrenal glands or make them more likely to spread or come back after treatment.

These same genetic changes may also be linked to other conditions, including neurofibromatosis type 1, multiple endocrine neoplasia type 2 (MEN2), Von Hippel-Lindau disease, and certain pancreatic neuroendocrine tumors.

At Penn Medicine, we offer genetic testing and counseling for people with pheochromocytomas and related conditions. Understanding genetic risk can help identify tumors earlier and guide screening and treatment plans for individuals and their families.

Our team includes doctors with deep experience in treating these rare tumors. Each case is reviewed by a tumor board through our Neuroendocrine Tumor Program, where experts develop a personalized treatment plan. We care for about 200 people with pheochromocytomas and paragangliomas each year.

In our program, you’ll find:

• Experienced care team: Many of our doctors are national leaders who also teach and lead research.
• Advanced treatments: We offer access to clinical trials and study new approaches, especially for tumors that spread.
• Complex surgical care: This includes procedures that may preserve part of the adrenal gland when possible.
• Recognized expertise: We are a Center of Excellence designated by the Pheo Para Alliance, a leading advocacy and research organization, and one of a small number of centers worldwide with this recognition.
• Ongoing support: A nurse coordinator helps schedule visits, answer questions, and guide care.