Pheochromocytoma and Paraganglioma Endocrine Tumors Everyday Health MenuNewslettersSearch Cancer Pheochromocytoma and Paraganglioma Endocrine Tumors By Julie Lynn MarksMedically Reviewed by Conor Steuer, MDReviewed: September 20, 2022Medically ReviewedSurgery is the optimal treatment for both pheochromocytomas (adrenal gland tumors) and paragangliomas (nerve cell tumors).Prasit Rodphan/ShutterstockPheochromocytomas and paragangliomas are endocrine tumors, which means they grow in parts of the body that produce hormones. (1) These tumors can form spontaneously, for no known reason, or they may develop because of a faulty gene (mutation). Pheochromocytomas and paragangliomas are usually benign (noncancerous), but they can be malignant (cancerous) and spread to other parts of the body. The good news is that there are a number of effective ways to treat these tumors and alleviate symptoms. (2) Most Recent in Cancer The Price of Being Invisible How One Black Woman Fought for Her Lung Cancer Diagnosis Types of Treatments for Cancer Standing Up for Better Lung Cancer Care New Guidelines Recommend Best Uses of Acupuncture Massage and Other Non-Drug Treatments for Cancer Pain What Is a Pheochromocytoma A pheochromocytoma is a tumor that forms in one or sometimes both of the two adrenal glands, located on top of the kidneys. The adrenal glands make hormones like adrenaline and noradrenaline, which are crucial to the body’s fight-or-flight response to stress. (2,3) Pheochromocytomas can cause the adrenal gland to produce too many hormones, leading to high blood pressure and a variety of other symptoms. (4) Tumors are usually benign, but about 1 in 3 are cancerous and may metastasize (spread) to other parts of the body. (5) Pheochromocytomas are rare, with one rough estimate suggesting only 8 of 1 million people have pheochromocytoma. (6) This type of tumor most often affects people who are between 20 and 50 years old, but pheochromocytomas can develop in patients of any age. (7) Symptoms of Pheochromocytoma Signs of a pheochromocytoma may include:High blood pressureSweatingTremorsRapid heartbeatHeadachePaleness in the faceShortness of breathAnxietyWeight lossConstipation (3) Triggers of Pheochromocytoma Symptoms of a pheochromocytoma may be constant, begin suddenly without warning, or be triggered by one of the following:Physical activityStress or anxietyA change in body positionChildbirthSurgery Foods or drinks containing high levels of a substance called tyramine (a naturally occurring amino acid) can also cause flare-ups. Cheese, chocolate, smoked meat, wine, and beer can all be problematic. Drugs such as monoamine oxidase inhibitors (MAOIs, used to treat depression) and stimulants can also bring on symptoms. (3) Diagnosing Pheochromocytoma Doctors may be able to detect a pheochromocytoma by performing one or more of the following tests:Blood testsUrine testsImaging tests, such as a computerized tomography (CT) scan, magnetic resonance imaging (MRI), M-iodobenzylguanidine (MIBG) scan, or positron emission tomography (PET) scan (3) Treatment Options for Pheochromocytoma Surgery to remove the tumor and the affected adrenal gland is the preferred treatment for pheochromocytomas, although in unusual instances surgeons may take out just the tumor and leave the adrenal gland in place. Doctors usually perform this operation laparoscopically, making a few tiny incisions in the skin and inserting a long, thin, wand-like device containing a video camera and small surgical tools. Laparoscopy is much less invasive than traditional surgery. Patients usually receive medication before surgery to lower the risk of developing dangerously high blood pressure while on the operating table. After surgery, the remaining adrenal gland compensates for the one that was removed, and blood pressure typically returns to normal. If the tumor is cancerous, patients may receive additional treatments, such as:Chemotherapy Also known simply as chemo, this treatment uses powerful drugs to kill cancer cells.Radiation Treatment Radiation delivered to a specific site in the body zaps cancer cells.Targeted Therapies Cutting-edge medicines zero in on specific cancer cell targets. (3) Editor s Top Picks on Rare Cancers 10 Essential Facts About Rare Diseases What I Learned When My Husband Got a Rare Cancer Speaking Cancer A Glossary of Formal and Informal Terms Used to Describe Cancer Tests Treatment Patients and More The Price of Being Invisible How One Black Woman Fought for Her Lung Cancer Diagnosis What Is a Paraganglioma A paraganglioma (also known as an extra-adrenal pheochromocytoma) is a tumor that develops in certain nerve cells located throughout the body. Paragangliomas are most commonly found in the abdomen but can grow in the skull area, neck, chest cavity, pelvis, or bladder. (8) In about one-half of all cases, paragangliomas produce too much of the hormone adrenaline, which can cause high blood pressure and other symptoms. Doctors in the United States diagnose paragangliomas in only about 2 per 1 million people each year. (9) These tumors can be benign or cancerous, but doctors can’t necessarily tell with a biopsy (looking at a sample of cells under a microscope). The only way to make a definitive cancer diagnosis is to see if cells have spread to other areas of the body. (9) Paragangliomas can affect anyone at any age but most often appear in people who are between 30 and 50 years old. (10) Types of Paraganglioma Including Glomus Jugulare Tumors There are a number of different types of paraganglioma, classified by their location in the body. A glomus jugulare tumor is a type of paraganglioma that forms near the base of the skull, in an area called the jugular foramen. (11) These tumors are almost always benign, but they can spread and cause symptoms, such as:DizzinessHearing problemsDifficulty swallowingPainWeakness in the facial muscles (12) Symptoms of Paraganglioma Signs of a paraganglioma may include:High blood pressureRapid heartbeatHeadacheFlushingSweatingAnxietyDifficulty breathingWeaknessPale skinBlurred visionWeight lossAbdominal painHigh blood sugarConstipation (9) Diagnosing Paraganglioma Doctors may be able to diagnose a paraganglioma by performing:Blood testsUrine testsImaging tests, such as a CT scan, MRI, or PET scan (10) Treatment Options for Paraganglioma When treating a patient with a paraganglioma, doctors might first try to get blood pressure under control with drugs known as alpha-blockers and beta-blockers. Surgery to remove the tumor is usually considered the optimal approach. Prior to the procedure, patients might take medication to counteract the effects of certain hormones produced by the tumor. Surgeons can remove most paragangliomas using minimally invasive laparoscopic methods. (9) For very slow-growing tumors, physicians may opt for a watch-and-wait approach, holding off on surgery for as long as possible while regularly monitoring the patient. Other treatments for a paraganglioma might include radiation therapy, which can help control tumor growth, and thermal ablation therapy, which involves destroying tumor deposits with heat. (10) Treatment Options for Glomus Jugulare Tumors Doctors typically remove glomus jugulare tumors (paragangliomas near the base of the skull) by performing one of the following procedures:Craniotomy This surgery involves removing part of the skull in order to access the brain.Endoscopic Endonasal Surgery In this procedure, surgeons operate on areas of the brain and spine by inserting a long, thin device called an endoscope through an incision in the back of the nose. If surgery isn’t an option, doctors may turn to a treatment called stereotactic radiosurgery that uses focused radiation beams to destroy tumors. (11) RELATED: Cancer: What Are the Treatment Options for Me? Paraganglioma vs Pheochromocytoma What s the Difference Both paragangliomas and pheochromocytomas are rare tumors that develop in hormone-producing tissue. A pheochromocytoma is considered a type of paraganglioma (13) A key difference is that pheochromocytomas are tumors that grow in the adrenal glands, while paragangliomas are tumors that develop outside the adrenal glands. Both types of tumors can cause similar symptoms and are often treated the same way. (2) Risk Factors for Pheochromocytomas and Paragangliomas Certain genetic disorders can increase the risk of developing a pheochromocytoma or paraganglioma. These include:Multiple Endocrine Neoplasia, Type 2 (MEN 2) This condition causes one or more of the endocrine glands to become overactive or form a tumor.Von Hippel-Lindau Disease In this condition, tumors and cysts form in different areas of the body.Neurofibromatosis 1 (NF1) This disorder results in changes in skin coloring and tumor growth along nerves in the skin, brain, and other parts of the body. (3,14) What Is Hereditary Paraganglioma-Pheochromocytoma When the genetic changes (mutations) that cause paragangliomas and pheochromocytomas are passed on from parents to children, the condition is known as hereditary paraganglioma-pheochromocytoma. (15) Researchers suspect that about half of pheochromocytomas are hereditary. (16) The mutations involved with hereditary paraganglioma-pheochromocytoma may affect one of the following genes:SDHASDHAF2SDHBSDHCSDHDTMEM127MAX (15) Men and women with hereditary paraganglioma-pheochromocytoma have a fifty-fifty chance of passing the gene mutation to their offspring. (15) Hereditary Paraganglioma-Pheochromocytoma Risks People born with hereditary paraganglioma-pheochromocytoma face an increased risk of developing tumors as they age. By age 30, between 20 and 50 percent of those with the syndrome develop at least one tumor. By age 50, that number goes up to 86 percent. This inherited syndrome also increases the risk of developing other types of tumors, such as:Gastrointestinal stromal tumors (GIST) in the digestive tractRenal cell carcinoma in the kidneyPapillary thyroid carcinoma in the thyroid gland (15) Diagnosing Hereditary Paraganglioma-Pheochromocytoma In order to make a diagnosis of hereditary paraganglioma-pheochromocytoma, doctors will ask a patient about symptoms and family history. The next step might be DNA testing of a blood sample to look for a telltale gene mutation. A genetic counselor can discuss the pros and cons of genetic testing and help put any findings in context. (14) Screening for Hereditary Paraganglioma-Pheochromocytoma For patients with this inherited syndrome, doctors will likely recommend regular screenings to spot any tumors that might develop as early as possible. But because this condition is rare, there’s no consensus about how often to screen. Many experts recommend screening children beginning at age 10, or at least 10 years before the earliest age any family members developed tumors. Doctors at St. Jude’s Children’s Research Hospital recommend the following protocol:Annual Physical Exams These should be performed by a doctor who is familiar with the condition.Annual Blood Tests High levels of certain hormones in the blood can be a red flag.Magnetic Resonance Imaging (MRI) These scans should be performed every one to two years to check for tumors. (15) Symptoms of Hereditary Paraganglioma-Pheochromocytoma Symptoms of hereditary paraganglioma-pheochromocytoma may include:High blood pressureHeadacheSweatingFast or irregular heartbeatAnxietyPale skinHearing lossTrouble swallowingCoughingHoarseness (15) Inheritance Patterns of Hereditary Paraganglioma-Pheochromocytoma The inheritance pattern of paraganglioma-pheochromocytoma syndrome is known as autosomal dominant. That means people who inherit an abnormal gene from one parent (not necessarily both) are at increased risk. (17) But developing hereditary paraganglioma-pheochromocytoma also requires a second mutation, one affecting the normal copy of the gene. This second, so-called somatic mutation develops during a person’s life and only appears in tumor cells. (13) Some types of hereditary paraganglioma-pheochromocytoma are passed on only if the mutated copy of the gene is inherited from the father, while other types can be inherited from either the father or mother. (13,15) RELATED: Cancer Risks, Signs, Symptoms, Tests, Treatments, and More NEWSLETTERS Sign up for our Cancer Care Newsletter SubscribeBy subscribing you agree to the Terms of Use and Privacy Policy. Editorial Sources and Fact-Checking ReferencesNeuroendocrine Tumors: Introduction. American Society of Clinical Oncology. January 2021.Pheochromocytoma and Paraganglioma Treatment (PDQ) — Patient Version. National Cancer Institute. January 7, 2022.Pheochromocytoma. Mayo Clinic. May 21, 2022.Pheochromocytoma. MedlinePlus. June 13, 2016.Pheochromocytoma and Paraganglioma: Condition Information. National Institute of Child Health and Human Development. December 1, 2016.Pheochromocytoma. National Cancer Institute. February 12, 2020.What Is Pheochromocytoma? Pheo Para Alliance.Paraganglioma: Diagnosis and Treatment. American Association of Endocrine Surgeons.Columbia Adrenal Center: Paraganglioma. Columbia University Irving Medical Center.Neuroendocrine Tumors: Paraganglioma. Mayo Clinic. January 11, 2022.Glomus Jugulare Tumors. Columbia University Irving Medical Center.Glomus Jugulare Tumors. National Institutes of Health: Genetic and Rare Diseases Information Center. November 8, 2021.Hereditary Paraganglioma-Pheochromocytoma. MedlinePlus. November 1, 2016.Hereditary Paraganglioma-Pheochromocytoma Syndrome. Children’s Hospital of Philadelphia.Hereditary Paraganglioma-Pheochromocytoma Syndrome. St. Jude’s Children’s Research Hospital. June 2020.Ask the Cancer Genetics Team: Inherited Tendency for Pheochromocytomas. Dana-Farber Cancer Institute.Autosomal Dominant. MedlinePlus. January 6, 2022.Show Less The Latest in Cancer New Guidelines Recommend Best Uses of Acupuncture Massage and Other Non-Drug Treatments for Cancer PainLeading organizations say complementary therapies such as reflexology and hypnosis may provide pain relief for some cancers.By Susan K. TreimanOctober 4, 2022 Is Chemotherapy Bringing You Down Research shows that chemotherapy can cause depression. 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