Pheochromocytomas (also known as "pheos") are rare neuroendocrine tumors that require expert care from a group of providers. While many pheochromocytomas are treatable with surgery, they occasionally need several forms of therapy.
Not many programs have the expertise to treat these challenging tumors. But at Penn Medicine, our team can accurately identify pheochromocytomas and find the most appropriate treatment for you. We provide all the support you need, including genetic services, with a focus on your quality of life.
What Is a Pheochromocytoma?
A pheochromocytoma, or a "pheo," is a type of neuroendocrine tumor found in adrenal glands. These two glands sit above the kidneys. They make the hormones epinephrine (adrenaline) and norepinephrine (noradrenaline), which control heart rate and blood pressure.
Pheochromocytomas develop in the center of the adrenal gland, an area called the adrenal medulla. They form in chromaffin cells — the same cells involved with many paragangliomas ("paras"), related neuroendocrine tumors found in other body areas. They can interfere with hormone levels in dangerous ways.
Most pheochromocytomas grow slowly, and many are benign (noncancerous). Less often, pheos are malignant (cancerous). Doctors see such cases more commonly in people with inherited genetic mutations placing them at higher risk for pheos and paras. Some people develop both pheos in the adrenal gland and paras in other parts of the body.
Even if they don't turn cancerous, most pheochromocytomas still require treatment.
What Are the Signs and Symptoms Associated with Pheochromocytoma?
Sometimes pheochromocytoma symptoms aren't very noticeable. More often, the tumors cause a classic set of signs that typically last for 15 to 20 minutes at a time. They may occur up to several times a day and include:
- Headaches
- Heart palpitations, marked by pounding, fluttering or beating rapidly
- Hypertension (high blood pressure), though levels may rise and fall and sometimes even drop too low
- Pale appearance
- Sweating
- Tremors, or shaking
The signs are tied to the adrenaline and noradrenaline chromaffin cells make. Those hormones (known as catecholamines) are neurotransmitters, sending signals from nerves to the heart and blood vessels. Pheochromocytomas often cause the adrenal glands to release more catecholamines than normal, raising blood pressure and heart rate.
Less commonly, pheochromocytomas can cause:
- Anxiety, nervousness or panic attacks
- Pain in the stomach
- Unintended weight loss
Pheochromocytoma Risk Factors and Genetics
We often don't know why pheochromocytoma forms. For the most part, researchers haven't identified factors that might raise the risk of developing one.
Up to 40 percent of pheochromocytomas result from genetic mutations (also known as variants) inherited from parents. If either parent carries a known variant, you have a 50-50 chance of inheriting it. But inheriting one of these genes doesn't guarantee you'll develop pheochromocytoma. The chances vary depending on the specific variant and other factors.
In addition to causing pheochromocytoma, mutations can also influence how the disease behaves. Some mutations (in RET, VHL, NF1 and TMEM127 genes) can cause tumors in both adrenal glands. And some mutations can make tumors more likely to turn cancerous and more likely to return after treatment.
These variants can also cause other rare tumors in the nerves, eyes, skin, uterus, kidneys, thyroid, pancreas, brain and spinal cord. Some variants cause larger syndromes with additional symptoms and medical conditions.
Genetic mutations involved include:
We offer genetic testing for neuroendocrine tumors as well as genetic counseling for individuals and families affected by pheochromocytomas and related conditions. Identifying risk helps us create personalized screening plans to find the disease early and make successful treatment more likely.
Pheochromocytoma Diagnosis
Most people are diagnosed with pheochromocytoma between the ages of 30 and 50. Sometimes, a pheochromocytoma is found during a scan given for another reason. More often, people come to us with symptoms or known genetic risks.
Unfortunately, many of the people who come to us have spent several years seeking an accurate diagnosis. Pheochromocytoma symptoms overlap with many other conditions, and they find that knowledge of the tumor varies.
At Penn, our team is well-equipped to make that evaluation. We recommend genetic testing for all people with pheochromocytoma to identify which variants may be involved. Results help us determine which scans and treatments to recommend.
Pheochromocytoma Testing
In addition to genetic testing, our evaluation typically includes:
- Family health history: We ask whether relatives have had a pheochromocytoma or a related tumor or are known to carry a connected genetic mutation.
- Blood or urine testing: We look for markers in the blood and urine left behind by catecholamines.
- Imaging: Scans help us determine the number of tumors, the location and the size. We start with either CT (X-rays from multiple angles) or MRI (using radio waves and a computer). If there's a chance of multiple tumors or cancer that has metastasized (spread), we also use a special scanning approach called functional imaging. This nuclear medicine tool shows tumors' behavior and uses molecules with low radiation.
Treating Pheochromocytoma: Our Team Approach
At Penn, you benefit from the experience and skill of an entire team. Every pheochromocytoma case goes before our tumor board, where experts from a range of fields come together and consider all options. Together, we create a personalized plan that balances the risks and benefits of treatment and considers your quality of life.
Care may involve:
Blocking Catecholamines
For pheochromocytomas that release adrenaline or noradrenaline, we use medications to block the hormones before any medical procedure. It prevents them from interfering with blood pressure to make treatment safer.
Members of our team have deep experience with these blockades, successfully performing them on more than 400 people. We tailor the medication choice and dose to each case. While most people only need blockers short-term, others may need them longer, such as those with metastatic disease and numerous symptoms.
Surgery to Remove Pheochromocytomas
We always want to remove the diseased adrenal gland when we can — it stops the extra hormone release, relieves symptoms and may cure the cancer. We may also need to remove the thyroid gland. Because pheochromocytomas tend to grow slowly, neuroendocrine tumor surgery is an option for many people.
Our surgeons typically take a minimally invasive approach to adrenal removal, performing surgery through tiny incisions. This approach requires just an overnight hospital stay, and you recover more quickly than with traditional open surgery.
The one remaining adrenal gland is usually able to produce enough hormones for normal body functions. But in rare cases tied to some genetic mutations, people have tumors in both glands.
In those cases, our surgeons may consider leaving the cortex, the outer part of the adrenal gland. This cortical-sparing surgery can particularly help younger people avoid taking adrenal-replacement pills long-term.
Treating Metastatic Pheochromocytomas
Pheochromocytoma occasionally comes back years after treatment. It can reappear in the lymph nodes, bones, liver or lungs. In other cases, pheochromocytoma has already metastasized by the time of initial diagnosis.
In either situation, we can help. Typically, the disease still grows more slowly and acts less aggressively than other cancers, so our team can have a chance to weigh the options:
- Surgery: We may recommend another tumor removal, especially if the disease is still limited to a few locations.
- Watchful waiting: Because pheochromocytoma is so slow-growing, we may recommend waiting to see how the cancer continues to act before deciding on a treatment.
- Ablation or radiation therapy: If metastases are confined to just a few small areas, we can use ablation (heat or cold therapy) to destroy tumors or radiation therapy to halt tumor growth.
- Systemic therapy: We may eventually consider treatment aimed at all cancerous cells. While these therapies don't always prompt a strong response and come with side effects, they can make a positive contribution. We carefully weigh the benefits and the downsides with you. We're also careful to not use up options too soon. Many people eventually try multiple therapies, going months or years between treatments. Even with metastatic disease, many people enjoy a good quality of life for a long time. In addition to clinical trials, treatment may include:
- Drug therapies: Injectable drugs that mimic hormones can relieve symptoms. Chemotherapy can also keep cancer in check, with newer, pill-based regimens that can feel less burdensome. And targeted therapy, a newer approach under development, aims at the ways cancer grows. Learn more about drug therapy for neuroendocrine tumors.
- Nuclear medicine: We pair controlled radioactive particles with substances that certain pheochromocytomas take into their cells. Our program led the national trial for Azedra, the only approved nuclear medicine therapy for pheochromocytoma. Other options are under study. Learn more about nuclear medicine for neuroendocrine tumors.
Lifelong Care for Pheochromocytomas
People with pheochromocytomas require lifelong checkups with scans and blood or urine tests, even if they underwent successful surgery. Our team creates a personalized plan based on your circumstances.
Frequency and imaging choice are based on factors such as whether you have an inherited genetic variant (and which one). Many people with variants need a full-body, rapid MRI, a method we developed at our program.
Why Choose the Abramson Cancer Center for Pheochromocytomas?
We bring together a full range of specialists, providing care through a focused program within the larger Penn Neuroendocrine Tumor Program. Our providers hold deep experience, seeing 200 people with paragangliomas and pheochromocytomas each year.
At our program, you'll find:
- Collaboration: Our providers work together to find the best plan, and most join the weekly neuroendocrine tumor board. Learn more about our tumor board and Neuroendocrine Tumor Program.
- Expertise: Our team specializes in pheochromocytoma, with years of experience treating these tumors. Many of our providers are nationally recognized experts, publishing research and giving talks about pheochromocytomas genetics, nuclear medicine and blood pressure control. Meet our neuroendocrine tumor team.
- Innovation: We run clinical trials to find new therapies, particularly for pheochromocytomas that metastasize (spread). Our ongoing project to analyze tumor samples is finding more clues about these challenging tumors and additional treatment targets. Learn more about neuroendocrine tumor research and clinical trials.
- Options: We provide highly technical treatments not widely available, including surgery that can spare a section of the adrenal gland when needed.
- Top recognition: We hold a Center of Excellence designation from the Pheo Para Alliance, the leading advocacy and research organization for pheochromocytomas and paragangliomas. It recognizes our personalized care, range of specialists and research projects. We're one of only nine such centers in the world, seven in the U.S. and the only program east of the Appalachians.
- Support: A nurse coordinator helps answer your questions, schedule appointments and attend to other needs. We remove as much stress and difficulty as we can. Throughout your treatment, we offer a range of additional support for neuroendocrine tumors.
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