Definition
Pheochromocytoma is a tumor that grows on the adrenal glands, which are located on top of the kidneys. 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
90% of these tumors are benign. These tumors occur elsewhere in the body and are called paragangliomas.
Causes
In most cases, pheochromocytoma has no known cause. In others, it is caused by a genetic mutation.
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
Many people don't have symptoms. In people who have them, pheochromocytoma may cause:
- High blood pressure, which can be constant or sporadic
- Severe headaches
- Excessive sweating
- Warmth, flushing
- Fast heart rate and pounding heart beat
- Sensation of a panic attack
- Blurred vision
- Nausea, vomiting, constipation or diarrhea
- Chest pain
- Involuntary trembling
- Pain in the lower chest or upper abdomen
- Increased appetite
- Weight loss
- Excessive thirst and urination
- Insomnia
- Tingling, burning, or numbness in the legs and feet
- Shortness of breath
- Muscle weakness
- Anxiety
- Unable to cope with high temperature
Diagnosis
You will be asked about your symptoms and medical history. A physical exam will be done.
Your doctor will need to test your body fluids for metanephrines. This can be done with:
- Urine testing
- Blood testing
A clonidine suppression test may be done if these tests do not confirm the diagnosis
Your doctor may need images of your bodily structures to look for the tumor and check if it has spread. These can be done with:
- CT scan
- Ultrasound
- MRI scan
- MIBG scintiscan, or adrenal medullary imaging
- PET scan
Treatment
Laparoscopic Adrenalectomy
The tumor will be removed whether it is cancerous or not. 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.
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.
Revision Information
- Reviewer: EBSCO Medical Review Board Michael Woods, MD, FAAP
- Review Date: 09/2018 -
- Update Date: 05/11/2013 -