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Pheochromocytoma
Sunday, 31 August 2003
Tuesday, 20 January 2004

What

Pheochromocytoma is a rare type of cancer (tumor) that grows in chromaffin cells of the adrenal glands. The human body has two adrenal glands that are located on top of each kidney. Pheochromocytoma grows in the center part of the adrenal gland, called the adrenal medulla. This part of the gland produces hormones, called catecholamines, which control many functions of the body including heart rate and blood pressure. The most well known of the catecholamines is epinephrine (adrenaline), which has the vast majority of its effects on the cardiovascular system. Not surprisingly, the symptoms associated with pheochromocytomas are due to an excess of the hormones epinephrine and norepinephrine, both potent stimulators of the cardiovascular system. Although tumors usually grow in only one of the adrenal glands, they can also grow in both glands and other areas of the body. There are two general types of pheochromocytoma, the most common type (about 90% of cases) remains in the area where it originally developed and is called benign. The less common type (about 10 % of cases) spreads to other parts of the body and is called malignant. If the tumor is malignant, it is important to get treatment as soon as possible so that it does not spread and destroy other organs of the body.

Who

Pheochromocytomas can occur at any age although they usually develop in young to middle-aged adults between the ages of 30 to 60. About ten percent of affected individuals, however are children who usually begin to have symptoms between the ages of 6 and 14. There is a slight predominance of males affected.

Signs and Symptoms

Symptoms of pheochromocytoma may include: 1. High blood pressure 2. Severe headache 3. Palpitations (racing of the heart) 4. Sweating 5. Chest pain 6. Abdominal pain 7. Flushing 8. Nervousness 9. Irritability 10. Increased appetite 11. Weight loss 12. Tremors 13. Nausea 14. Vomiting Commonly, a person with pheochromocytoma will have attacks of these symptoms (paroxysms) at unpredictable times. These paroxysms are caused by a sudden release of catecholamines. The attacks usually include high blood pressure and can last for 15 minutes to an hour. High blood pressure may be sustained (hypertension) or intermittent.

Possible Causes

Both environmental and genetic factors are thought to cause most cases of pheochromocytoma although the exact causes are not known . In many cases, however the tumor develops without any known family history of cancer. Often, the condition occurs in association with another disorder. These “associated” disorders include: neurofibromatosis, Von Hippel-Lindau disease, multiple endocrine neoplasia (MEN) syndromes, tuberous sclerosis, Sturge-Weber syndrome, and ataxia-telangiectasia.

Diagnosis

Pheochromocytoma can be diagnosed by testing the levels of catecholamines or their break-down products present in the individual. A blood or urine test can determine this. Also, imaging tests can be done to take pictures of the inside of the body to see if a mass (that could be a tumor) is present. These imaging tests include: Magnetic Resonance Imaging (MRI), Computed Tomography (CT Scan), MIBG (metaiobenzylguanidine) scan. In addition, a small piece of adrenal gland tissue can be removed (adrenal biopsy) and examined for cells indicative of pheochromocytoma.

Treatment

Treatment for pheochromocytoma usually involves medications that lower blood pressure and surgery to remove the tumor(s). Once the diagnosis is certain, the patient is treated with medications that counteract the effect of the catecholamines. Treatment last for a number of days prior to surgery. For those with malignant tumors, treatment with medications and/or radiation that kills the cancer cells may also be necessary. The exact type of treatment depends on factors such as the child’s age, health status, medical history, the extent of the disease, and the type of pheochromocytoma.

Prognosis

For those with benign pheochromocytoma, the chance of full recovery, with no recurrence of tumor growth or symptoms is very high. Recovery for those with malignant pheochromocytoma depends on the how far the cancer has spread, the person’s age, and health status. For those patients in whom the tumor is not amenable to surgical excision medical management can prolong survival for a number of years.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

National Institutes of Health
Easy to understand information for patients and families.

National Cancer Institute
A good source of cancer-related information for patients and families. This site also contains links to pertinent clinical trials and current research.

Pheochromocytoma Group Support Site
A nice website dedicated to providing support for those affected by the disease.

Google Search for Pheochromocytoma

References and Sources

www.cc.nih.gov/ccc/patient_education/pepubs/pheo.pdf www.cancer.gov/cancerinfo/pdq/treatment/pheochromocytoma/patient/ www.nlm.nih.gov/medlineplus/ency/article/000340.htm home.mdconsult.com/das/book/29127195/view/897?sid=186515713 www.lpch.org/DiseaseHealthInfo/HealthLibrary/diabetes/pcm.html