Pheochromocytoma

Title

How can we make GARD better? We want to hear from you.
Provide Feedback

Other Names:

Adrenal Gland Chromaffin Paraganglioma; Adrenal Gland Chromaffinoma; Adrenal Gland Paraganglioma; Adrenal Gland Chromaffin Paraganglioma; Adrenal Gland Chromaffinoma; Adrenal Gland Paraganglioma; Intraadrenal Paraganglioma; Chromaffin Paraganglioma of the Adrenal Gland; Adrenal Gland Pheochromocytoma See More

Categories:

Blood Diseases; Congenital and Genetic Diseases; Endocrine Diseases; Blood Diseases; Congenital and Genetic Diseases; Endocrine Diseases; Hereditary Cancer Syndromes; Kidney and Urinary Diseases; Rare Cancers See More

This disease is grouped under:

Neuroendocrine tumor

Summary Summary

Pheochromocytomas are tumors of the adrenal glands. These glands are located right above the kidneys. Pheochromocytomas cause the adrenal glands to make too many stress hormones called epinephrines and norepinephrines. This can lead to high blood pressure and cause symptoms such as severe headaches, irritability, sweating, rapid heart rate, nausea, vomiting, weight loss, weakness, chest pain, and anxiety. Rarely, this kind of tumor occurs outside the adrenal gland, usually somewhere in the abdomen. These are called extra-adrenal pheochromocytomas or paragangliomas. The cause of most pheochromocytomas is unknown. In some cases, there is a genetic cause. This type of tumor can occur in certain familial genetic syndromes, including multiple endocrine neoplasia, type 2 (MEN2), neurofibromatosis type 1, Von Hippel-Lindau disease, hereditary paraganglioma-pheochromocytoma syndrome, Carney triad, and Carney-Stratakis dyad. There are also several genes that have been associated with pheochromocytoma when it does not occur as part of a syndrome.^[1]^[2]

Last updated: 2/7/2018

Diagnosis Diagnosis

Making a diagnosis for a genetic or rare disease can often be challenging. Healthcare professionals typically look at a person’s medical history, symptoms, physical exam, and laboratory test results in order to make a diagnosis. The following resources provide information relating to diagnosis and testing for this condition. If you have questions about getting a diagnosis, you should contact a healthcare professional.

Testing Resources

The Genetic Testing Registry (GTR) provides information about the genetic tests for this condition. The intended audience for the GTR is health care providers and researchers. Patients and consumers with specific questions about a genetic test should contact a health care provider or a genetics professional.

Do you have updated information on this disease? We want to hear from you.

Treatment Treatment

The recommended treatment of a pheochromocytoma is removal by surgery, even in cases where there are pheochromocytomas in both adrenal glands.^[3] Prior to surgery, it is important to take medications to control and stabilize blood pressure. Following surgery, affected individuals should visit their physicians regularly to monitor blood pressure and blood levels of metanephrine.^[1]^[4] Long-term treatment with medication should only be considered if, for some reason, the pheochromocytoma cannot be removed by surgery.^[1]

Last updated: 8/24/2014

Management Guidelines

The North American Neuroendocrine Tumor Society has published several consensus guidelines relating to the medical treatment and management of neuroendocrine tumors. Guidelines are developed pursuant to National Institute of Health (NIH) standards and serve as important references for practicing physicians.

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Iobenguane I 123 (Brand name: Adreview™) - Manufactured by GE Healthcare, Inc
FDA-approved indication: To be used in the detection of primary or metastatic pheochromocytomas or neuroblastomas as an adjunct to other diagnostic tests
National Library of Medicine Drug Information Portal
Iobenguane I 131 (Brand name: Azedra) - Manufactured by Progenics Pharmaceuticals, Inc.
FDA-approved indication: July 2018, iobenguane I 131 (Azedra) was approved for the treatment of adult and pediatric patients 12 years and older with iobenguane scan positive, unresectable, locally advanced or metastatic pheochromocytoma or paraganglioma who require systemic anticancer therapy.
National Library of Medicine Drug Information Portal

Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

To find a medical professional who specializes in genetics, you can ask your doctor for a referral or you can search for one yourself. Online directories are provided by the American College of Medical Genetics and the National Society of Genetic Counselors. If you need additional help, contact a GARD Information Specialist. You can also learn more about genetic consultations from Genetics Home Reference.
The Neuroendocrine Tumor Research Foundation has a Doctor Database for help finding a physician specializing in treating neuroendocrine tumors. They also provide a link to a list of multidisciplinary diagnosis and treatment centers on the same web page.
The Pheo Para Alliance has organized a directory of physicians, surgeons, researchers, experts and others experienced in the treatment and management of pheochromocytoma and paraganglioma.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Pheochromocytoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

National Adrenal Diseases Foundation (NADF)
P.O. Box 566
Lake Zurich, IL 60047
Telephone: +1-(847) 726-9010
E-mail: nadfmail@nadf.us
Website: https://www.nadf.us
NET Patient Foundation (UK and Ireland)
Holly House (2nd Floor)
74 Upper Holly Walk
Leamington Spa
Warwickshire, CV32 4JL United Kingdom
Toll-free: 0800 434 6476 (Helpline)
Telephone: 01926 883487 (General inquieries)
E-mail: hello@netpatientfoundation.org
Website: https://www.netpatientfoundation.org/
Neuroendocrine Cancer Awareness Network (NCAN)
3074 Brookchase Blvd
Fort Mill, SC 29707
Telephone: 866-850-9555
E-mail: contact@netcancerawareness.org , help@netcancerawareness.org
Website: https://www.netcancerawareness.org/
North American Neuroendocrine Tumor Society (NANETS)
136 Everett Road
Albany, NY 12205
Telephone: (650) 762-6387
E-mail: staff@nanets.net
Website: https://nanets.net/
The Healing NET Foundation
200 Hill Avenue
Suite 4
Nashville, TN 37210
Telephone: 615-3696463
E-mail: info@thehealingnet.org
Website: https://www.thehealingnet.org/
The Pheo Para Alliance
9721 Whitley Park Place
Bethesda, MD 20814
E-mail: pheoalliance@gmail.com
Website: https://pheopara.org/

Organizations Providing General Support

American Cancer Society
250 Williams Street NW
Atlanta, GA 30329
Toll-free: 1-800-227-2345
Website: https://www.cancer.org
Cancer Hope Network
2 North Road, Suite A
Chester, NJ 07930
Toll-free: 1-877-467-3638 (1-877-HOPENET)
Telephone: +1-908-879-4039
Fax: +1-908-879-6518
E-mail: info@cancerhopenetwork.org/
Website: https://www.cancerhopenetwork.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD) provides information related to the health of children, adults, and families. Click on the link to view information on this topic.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The Merck Manuals Online Medical Library provides information on this condition for patients and caregivers.
The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.
The NET Patient Foundation has an online handbook that aims to serve as a guide to living with neuroendocrine tumors.
The Neuroendocrine Tumor Research Foundation provides information about Pheochromocytoma.

In-Depth Information

GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions.
Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
PubMed is a searchable database of medical literature and lists journal articles that discuss Pheochromocytoma. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Sweeney AT. Pheochromocytoma. eMedicine. June 2014; http://emedicine.medscape.com/article/124059-overview. Accessed 8/24/2014.
Salman Kirmani, MBBS and William F Young, MD, MSc. Hereditary Paraganglioma-Pheochromocytoma Syndromes. GeneReviews. November 2014; http://www.ncbi.nlm.nih.gov/books/NBK1548/.
Gumbs AA, Gagner M. Laparoscopic adrenalectomy. Best Practice & Research Clinical Endocrinology & Metabolism. 2006; 20(3):483-499. http://www.ncbi.nlm.nih.gov/pubmed/16980207. Accessed 8/24/2014.
National Comprehensive Cancer Network. Neuroendocrine Tumors. NCCN Clinical Practice Guidelines in Oncology. 2014; http://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Accessed 11/22/2014.