Pheochromocytoma and Paraganglioma

Pheochromocytomas (often abbreviated as pheos) and paragangliomas (often abbreviated as paras) are rare types of tumors. Pheochromocytomas form in the adrenal glands, while closely related paragangliomas originate from cells of neuronal origin, which can be located throughout the neck, chest, abdomen, or pelvis. These tumors can produce hormones that control the typical body stress reaction and other functions, including heart rate and blood pressure.

NICHD supports and conducts research on pheochromocytoma and paraganglioma to understand the causes of the disease, improve detection methods, and develop effective therapies.

On this webpage, the term “pheochromocytoma” also refers to paraganglioma unless otherwise specified. 

About Pheochromocytoma and Paraganglioma

Pheochromocytoma is a rare tumor that develops in the adrenal glands. There are two adrenal glands in the human body, located on top of the kidneys. Each adrenal gland has two parts, the outer cortex and inner medulla. The cortex produces corticosteroid and androgen hormones. The medulla produces catecholamines (epinephrine, norepinephrine, and dopamine).

Pheochromocytomas may be found in one or both glands and may spread, or metastasize, beyond the adrenal glands. Pheochromocytomas develop from the center of the adrenal gland, in an area called the adrenal medulla, which secretes catecholamines.1

Hormones that are produced typically by the adrenal medulla, catecholamines, help to regulate heart rate, blood pressure, and the body’s responses to stress. Pheochromocytomas release additional catecholamines, causing higher-than-usual amounts in the body.2 Changes in hormone levels produce some of the clinical signs and life-threatening symptoms of pheochromocytoma.

Although the majority of pheochromocytomas are benign (noncancerous or nonmetastatic), about one third are malignant (cancerous or metastatic) and spread to other parts of the body.3 Malignant pheochromocytomas may spread, or metastasize, to the liver, lungs, bones, and lymph nodes.2

On this webpage, the term “pheochromocytoma” also refers to paraganglioma unless otherwise specified.

Paragangliomas are tumors originating from neuronal tissue; they were formerly called extra-adrenal pheochromocytomas. Developmentally, there are two subgroups of these tumors: parasympathetic paragangliomas and sympathetic paragangliomas. These subgroups differ in the type of tissue from which they form (parasympathetic versus sympathetic) and also in their location and hormonal production. Parasympathetic tissue is important for certain body processes, including salivation, urination, and digestion. Sympathetic tissue forms the tissue important for fight-or-flight responses.

Paragangliomas that develop from parasympathetic-associated tissue in the head and neck are usually referred to as “head and neck paragangliomas.” These tumors can be locally invasive but usually do not metastasize or produce catecholamines (stress hormones). Signs and symptoms of head and neck paragangliomas are usually caused by the tumor mass itself instead of the secreted catecholamines.

Paragangliomas that develop from sympathetic neuronal tissue are usually localized in the chest, abdomen, or pelvis. These tumors often have excessive hormone secretion, which makes them very similar to pheochromocytomas. As a result of excessive hormone secretion, paragangliomas often cause the signs and symptoms described below (e.g., heart palpitations, irregular heartbeat, hypertension, sweating). Sympathetic (or extra-adrenal) paragangliomas generally tend to be more malignant than pheochromocytomas (localized to the adrenal gland).4


  1. National Cancer Institute, PDQ Cancer Information Summaries. (2012). Pheochromocytoma and paraganglioma treatment (PDQ®). Retrieved March 7, 2012, from 
  2. Pacak, K. (2011). Phaeochromocytoma: a catecholamine and oxidative stress disorder. Endocrine Regulation 45: 65-90.
  3. Parenti, G., Zampetti, B., Rapizzi, E., Ercolino, T., Giachè, V., & Mannelli, M. (2012). Updated and new perspectives on diagnosis, prognosis, and therapy of malignant pheochromocytoma/paraganglioma. Journal of Oncology, 2012, 872713.
  4. Eisenhofer, G., Lenders J. W., Siegert, G., Bornstein, S. R., Friberg, P., Milosevic, D., et al., (2012). Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. European Journal of Cancer, 48: 1739–1749.

What are common symptoms of pheochromocytoma?

Pheochromocytoma can cause a variety of signs and symptoms, depending on the type and location of the tumor.

Some of the most common signs and symptoms include (in alphabetical order):

  • Abdominal pain
  • Constipation
  • Chest pain
  • Dizziness
  • Elevated blood sugar
  • Facial flushing (redness)
  • High blood pressure
  • Increased respiratory rate
  • Nausea
  • Nervousness, anxiety, and irritability
  • Pale skin tone
  • Rapid heart rate and heart palpitations
  • Severe headaches
  • Sweating
  • Visual disturbances
  • Weight loss

What is the most common age for diagnosis of pheochromocytoma?

Pheochromocytoma can occur at any age. However, it is diagnosed most frequently between the ages of 30 and 50.1

Up to 20% of pheochromocytomas are diagnosed in children.2


  1. Eunice Kennedy Shriver National Institute of Child Health and Human Development. The proper diagnosis, treatment, genetics, and research of pheochromocytoma and paraganglioma: Overview. Retrieved March 16, 2012, from
  2. King, K. S., Prodanov, T., Kantorovich, V., Fojo, T., Hewitt, J. K., Zacharin, M., et al., (2011). Metastatic pheochromocytoma/paraganglioma related to primary tumor development in childhood or adolescence: significant link to SDHB mutations. Journal of Clinical Oncology 29: 4137-4142.

What causes pheochromocytoma?

Pheochromocytoma can be a genetic condition inherited from a parent, or it can show up in someone with no family history of the condition.

Approximately one third of pheochromocytoma cases occur when patients inherit a mutated gene from their parents.1 Studies have linked several genes to the disease, but researchers are not sure how these genes contribute to the formation of this tumor.2

The remaining two thirds of cases are spontaneous and are not associated with a family history. However, genetic inheritance may play a role in the development of the disease through unknown genes. For example, in one study, a significant percentage of patients (7.5%–27%) with sporadic pheochromocytoma had genetic mutations that have been linked to other family-inherited syndromes.3


  1. Eunice Kennedy Shriver National Institute of Child Health and Human Development. The proper diagnosis, treatment, genetics, and research of pheochromocytoma and paraganglioma: Genetic screening. Retrieved March 26, 2012, from
  2. Karasek, D., Shah, U., Frysak, Z., Stratakis, C., & Pacak, K. (2013). An update on the genetics of pheochromocytoma. Journal of Human Hypertension, 27, 141–147.
  3. Pacak, K., Lenders, J. W. M., & Eisenhofer, G. (2007). Pheochromocytoma: diagnosis, localization, and treatment. Malden, MA: Wiley-Blackwell.

How do health care providers diagnose pheochromocytoma?

A health care provider uses blood and urine tests that measure catecholamines and/or their metabolites to diagnose pheochromocytoma. Metabolites are biochemical substances that form when another substance is broken down in the body. Higher-than-typical amounts of these biochemical substances in the blood and/or urine can be an indication of the presence of a pheochromocytoma/paraganglioma.

Pheochromocytomas can secrete all, none, or any combination of catecholamines (epinephrine, norepinephrine, dopamine) and their metabolites, which are called metanephrines (metanephrine, normetanephrine, methoxytyramine). Multiple studies at the National Institutes of Health (NIH) have demonstrated the utility of measuring metanephrines in the blood for the diagnosis of pheochromocytoma/paraganglioma.1 This method is often recommended as the first diagnostic test when there is suspicion of a pheochromocytoma/paraganglioma.

Tumors can also be found accidentally during nonrelated imaging studies. The location of a pheochromocytoma can be determined by using several imaging methods, including computed tomography (CT) and magnetic resonance imaging (MRI). CT scans use X-rays to produce detailed images of the inside of the body, while MRI uses magnetic waves to produce these pictures. A third imaging method that can be used to detect pheochromocytomas is MIBG (metaiodobenzylguanidine) scintigraphy. During this procedure, MIBG, a compound containing a small amount of radioactivity, is injected into a vein and is picked up by pheochromocytoma cells but not healthy cells.1 The body is scanned with a scanner that detects the MIBG. Any MIBG that is seen with the scanner can indicate the presence of pheochromocytoma cells.

Read more about getting an MIBG scintiscan.


  1. Pacak, K., & Eisenhofer G. (2007). An assessment of biochemical tests for the diagnosis of pheochromocytoma. Nature Clinical Practice Endocrinology & Metabolism. 3: 744-745.

Is there a cure for pheochromocytoma?

The success of pheochromocytoma treatment depends upon several factors; the most important include the presence of metastasis, genetics, location, and overall extent of the disease. Malignant pheochromocytoma can only be determined by the presence of metastasis or tumor spreading (tumors in locations such as the bones, liver, lungs, or lymph nodes).

The only curative treatment for pheochromocytoma is the complete surgical removal of the tumor. The long-term prognosis of patients after resection of a single sporadic pheochromocytoma is excellent.

Even after tumor removal, there is still a risk that pheochromocytoma or paraganglioma might return (called recurrence). Recurrence is more likely for those who have paraganglioma, those who are younger, those whose family members also have the disease, and those who had large tumors.1

There is currently no cure for malignant pheochromocytoma. Radiotherapy, or the use of high-powered X-rays or radioactive beams to destroy tumors, can assist in shrinking some malignant tumors.2 Tumor shrinkage can lessen the clinical signs and symptoms of pheochromocytoma by reducing the production of hormones and in some cases may allow for surgery.

Although not curative, medications are used to control the clinical signs and symptoms of both benign and malignant pheochromocytoma.


  1. Amar, L., Lussey-Lepoutre, C., Lenders, J. W., Djadi-Prat, J., Plouin, P. F., & Steichen, O. (2016). Management of endocrine disease: Recurrence or new tumors after complete resection of pheochromocytomas and paragangliomas: a systematic review and meta-analysis. European Journal of Endocrinology, 175(4), R135–R145.
  2. Lenders, J. W. M., & Eisenhofer, G. (2017). Update on modern management of pheochromocytoma and paraganglioma. Endocrinology and Metabolism (Seoul, Korea), 32(2), 152–161.

What are the treatments for pheochromocytoma?

Standard treatments for pheochromocytoma include1,2:

  • Surgical removal of the tumor
  • Medications (chemotherapy) designed to kill tumor cells
  • Radiotherapy: using radio waves to destroy the tumors
  • Medications to control the signs and symptoms of the disease

Types of Therapies for Pheochromocytoma:

Ninety percent of patients are cured by surgery to remove benign pheochromocytoma tumors.3 Surgery for tumor removal is typically done by laparoscopy, during which a small incision is made in the abdomen.3,4 During surgery to remove the tumor, the physician will usually examine nearby organs to determine whether the pheochromocytoma has spread to other parts of the body.

Medications are prescribed to treat the clinical signs and symptoms of pheochromocytoma.

Commonly prescribed medications include the following1:

  • Alpha-adrenergic blockers to lower blood pressure
  • Beta blockers for controlling rapid, irregular pulse

For patients whose tumors are successfully removed, blood pressure and hormone levels usually return to expected levels over the weeks immediately following surgery.


  1. National Cancer Institute. (2012). Pheochromocytoma and paraganglioma treatment (PDQ). Retrieved March 7, 2012, from
  2. Waguespack, S. G., Rich, T., Grubbs, E., Ying, A. K., Perrier, N. D., Ayala-Ramirez, M., et al. (2010). A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. Journal of Clinical Endocrinology and Metabolism, 95(5), 2023–2037.
  3. National Organization for Rare Diseases (2011). Pheochromocytoma. external link
  4. Lentschener, C., Gaujoux, S., Tesniere, A., & Dousset, B. (2011). Point of controversy: Perioperative care of patients undergoing pheochromocytoma removal—time for a reappraisal? European Journal of Endocrinology, 165(: 365-373.
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