Pronounced: Fee-o-crome-o-sigh-toh-maEn Espaol (Spanish Version)
Pheochromocytoma is a tumor made up of special adrenal gland cells known as the chromaffin cells which secrete epinephrine, norepinephrine, and dopamine. These hormones are active in the regulation of heart rate and blood pressure. Pheochromocytoma cells periodically secrete excessive amounts of the hormones epinephrine and norepinephrine, resulting in periods of very high blood pressure , rapid heart beats or palpitations, excess sweating, and severe headaches.
Most pheochromocytoma are benign (noncancerous) and about 10% are malignant (cancerous). Most pheochromocytoma grow on the adrenal glands, which are themselves perched on top of the kidneys. About 10%-20% of all pheochromocytoma occur elsewhere in the body.
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The cause of pheochromocytoma is not yet known. Scientists suspect a genetic link.
A risk factor is something that increases your chance of getting a disease or condition.
Factors associated with pheochromocytoma include:
- A family history of pheochromocytoma, tumors in other glands of the body, or other hormonal disorders
- Genetic diseases including:
Up to 50% of patients have no symptoms. Pheochromocytoma is found by a CT scan or MRI scan during the investigation of some other unrelated illness.
Symptoms may occur many times during the day or as infrequently as once every few months. Symptoms may be brought on by pressure on the tumor (as might occur accidentally during a massage), medications (such as certain anesthetics and beta-blockers), or intense emotion. Symptoms can include: Severe headachesExcessive sweating Fast heart rate ( tachycardia ) Sensation of a panic attackVision changes (blurred vision)Nausea, vomiting, constipationPounding heart beat (palpitations)Chest painInvoluntary trembling (tremor)Pain in the lower chest or upper abdomenWarmth, flushingIncreased appetiteWeight lossInsomniaHigh blood pressure (either sporadic or constant)Tingling, burning, or numbness in the legs and feetShortness of breathMuscle weaknessAnxietyIntolerant to high temperature Diagnosis Your doctor will take a medical history and perform a physical exam. Diagnosis of pheochromocytoma may include the following tests: 24-hour urine testingto measure amounts of catecholamines (a group of hormones made by the adrenal glands near the kidneys), including epinephrine and norepinephrine and their byproducts (metanephrines)Blood testingto measure catecholamines and metanephrinesClonidine suppression testa dose of the drug clonidine is given, and blood levels of norepinephrine are tested CT scana type of x-ray that uses a computer to make pictures of structures inside the body The CT scan can be used to diagnose the presence of the tumor.Ultrasound a noninvasive test using sound waves, which may be used to help detect adrenal tumors MRI scana test that uses magnetic waves to produce cross-sectional or three-dimensional images of the bodys tissuesMIBG scintiscan (or adrenal medullary imaging)an imaging test for diagnosing adrenal tumors, in which tiny amounts of radioactive materials are injected into the body Treatment If pheochromocytoma is benign (not cancerous), then the tumor is surgically removed. Laparoscopic adrenalectomy is the surgical removal of the adrenal glands. It involves making three or four small incisions in the abdomen and inserting a special camera through the incisions so that the surgeon can see the tumor area.
Sometimes, the adrenal glands are removed as a part of this process. Prior to surgical removal, the high blood pressure will need to be brought under control, using first the medication phenoxybenzamine, followed by beta-blocking antihypertensive agents. If pheochromocytoma is cancerous and have spread outside of the adrenal glands, chemotherapy involving the use of docarbazine, vincristine, and cyclophosphamide could be tried. PreventionSince little is known about the causes of pheochromocytoma, prevention methods are not available. RESOURCES: National Cancer Institutehttp://www.cancer.gov/cancer CANADIAN RESOURCES: BC HealthGuidehttp://www.bchealthguide.org Canadian Cancer Societyhttp://www.cancer.ca References: Beers MH et al. The Merck Manual of Medical InformationHome Edition . 2nd ed. Simon and Schuster, Inc.; 2003. Ferri FF. Ferri's Clinical Advisor: Instant Diagnosis and Treatment . 8th ed. St. Louis: Mosby; 2006. Goldman L. Cecil Textbook of Medicine . 22nd ed. Philadelphia: Saunders; 2004. Lenders JW, Eisenhofer G, Mannelli M, et al. Phaeochromocytoma. Lancet . 2005 Aug 20-26;366(9486):665-75.
Mittendorf EA, Evans DB, Lee JE, et al. Pheochromocytoma: advances in genetics, diagnosis, localization and treatment. Hematol Oncol Clin North Am . 2007;21:509-25. National Cancer Institute website. Available at: http://www.cancer.gov/ . Accessed March 26, 2003. Widimsky J Jr. Recent advances in the diagnosis and treatment of pheochromacytoma. Kidney Blood Res . 2006;29:321-6 Last reviewed October 2007 by David Juan, MDPlease be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.