(Pheo; Adrenal Gland Tumor; Pheochromocytosis)
Definition
Definition
Pheochromocytoma is a tumor. It is made up of special adrenal gland cells. The cells secrete hormones such as epinephrine and norepinephrine. These hormones help to regulate the heart rate and blood pressure. The cells may secrete excessive amounts of the hormones. This results in periods of:
- Very high blood pressure
- Rapid heartbeat or palpitations
- Excessive sweating
- Severe headaches
Most of these tumors are benign and grow on the adrenal glands, which are located on top of the kidneys. Other tumors occur elsewhere in the body.
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Causes
Causes
In most cases, pheochromocytoma has no known cause. In others, it is caused by a genetic mutation.
Risk Factors
Risk Factors
Factors that may increase your chance of pheochromocytoma include:
- A family history
- Tumors in other glands of the body
- Hormonal disorders
Genetic diseases associated with pheochromocytoma include:
- Von Hippel-Lindau disease
- Multiple endocrine neoplasia, type 2
- Neurofibromatosis type 1
- Paraganglioma syndromes
Symptoms
Symptoms
Most people don’t have symptoms. In people who have them, pheochromocytoma may cause:
- Severe headaches
- Excessive sweating
- Fast heart rate
- Sensation of a panic attack
- Blurred vision
- Nausea, vomiting, constipation
- Pounding heart beat
- Chest pain
- Involuntary trembling
- Pain in the lower chest or upper abdomen
- Warmth, flushing
- Increased appetite
- Weight loss
- Insomnia
- High blood pressure, which can be constant or sporadic
- Tingling, burning, or numbness in the legs and feet
- Shortness of breath
- Muscle weakness
- Anxiety
- Unable to cope with high temperature
- Aggressive or unusual behavior
Diagnosis
Diagnosis
Your doctor will ask about your symptoms and medical history. A physical exam will be done.
Your doctor may need to test your body fluids. This can be done with:
- Urine testing
- Blood testing
- Clonidine suppression test
Your doctor may need images of your bodily structures. These can be done with:
- CT scan
- Ultrasound
- MRI scan
- MIBG scintiscan, or adrenal medullary imaging
- PET scan
Treatment
Treatment
Laparoscopic Adrenalectomy
If the tumor is benign, it is removed. This procedure uses small incisions and special instruments. Sometimes, the adrenal glands are removed as a part of this process.
Medications
Prior to surgery, high blood pressure will need to be brought under control using blood pressure lowering medications.
Chemotherapy and Radiation Therapy
Chemotherapy may be used if the tumor is cancerous and has spread outside of the adrenal glands.
Radiation therapy may also be used to help destroy the tumors if they have spread.
Prevention
Prevention
There are no current guidelines to prevent pheochromocytoma.
RESOURCES:
CANADIAN RESOURCES:
Public Health Agency of Canada http://www.phac-aspc.gc.ca
References:
Lenders JW, Eisenhofer G, Mannelli M, Pacak K. Phaeochromocytoma. Lancet. 2005;366(9486):665-675.
Mittendorf EA, Evans DB, Lee JE, Perrier ND. Pheochromocytoma: advances in genetics, diagnosis, localization and treatment. Hematol Oncol Clin North Am. 2007;21(3):509-525.
Pheochromocytoma and paraganglioma. EBSCO DynaMed website. Available at:
http://www.ebscohost.com/dynamed
Updated July 1, 2014. Accessed September 4, 2014.
Widimsky J Jr. Recent advances in the diagnosis and treatment of pheochromacytoma. Kidney Blood Res. 2006;29(5):321-326.
Last Updated: 5/11/2013