Phaeochromocytoma Treatment in France

Overview

Epinephrine, norepinephrine, and dopamine are secreted in the adrenal medulla and are referred to as catecholamines. They are also known as fight or flight hormones and are responsible for that feeling of a rush when in fear. These hormones control heart rate and metabolism. Pheochromocytomas may be large in size; however, most are smaller than 10 centimeters.

• Abdominal pain
• Chest pain
• Hand tremor
• Localized amyloid deposits
• High blood glucose levels
• High blood pressure
• Irritability, nervousness, and anxiety
• Pallor
• Rapid heart rate
• Severe headache
• Palpitations
• Excessive sweating
• Sleeping difficulty
• Skin sensations
• Weight loss

If you suspect pheochromocytomas you should see your doctor who will then perform a physical exam on you and also ask questions. He/she may also order for some tests to be done to confirm the diagnosis. This include:

• Abdominal CT scan
• Abdominal MRI
• MIBG scintiscan
• Glucose test
• Adrenal biopsy
• Serum catecholamines (Catecholamines blood test)
• Serum metanephrine (Metanephrine blood test)
• Urine catecholamines
• Urine metanephrines

Pheochromocytomas are usually treated with surgery or medications. However, Chemotherapy and radiation therapy are not effective in treating pheochromocytomas.

Pheochromocytoma is usually treated with surgery; either by open laparotomy or laparoscopy to remove the tumor. Your doctor will prescribe and administe certain medications before the surgery, to stabilize your pulse and blood pressure. This will be done in the hospital to enable the doctor to closely monitor your vital signs. After the surgery, you will be admitted to the intensive care unit (ICU) so that your doctor can continuously monitor your vital signs.It may be necessary for the surgeon to completely remove the affected adrenal glands; a procedure known as an adrenalectomy.

Because this surgical operation is complex and the risks of complications are high, it is highly advisable to have this surgery at medical centers that specialize in the management of this disorder. Such medical centers will have all the necessary resources readily available such as anesthesia and endocrine resources. The surgical option requires prior treatment with medications such as the non-specific and irreversible alpha-adrenoceptor blocker phenoxybenzamine or a short-acting alpha antagonist. This minimizes the likelihood of severe intraoperative hypertension which might occur as the surgery proceeds.

A patient with pheochromocytoma is usually volume depleted. This implies that blood volume is reduced due to an excessive fluid loss in the urine caused by almost total inhibition of renin-angiotensin activity. Therefore, once the pheochromocytoma has been resected, there is volume depletion and a very low sympathetic activity which can lead to profound hypotension. It is therefore, usually advised to ‘salt load’pheochromocytoma patients before their surgery. Your doctor will advise you to consume foods high in salt food pre-operatively. Direct salt replacement or the administration of intravenous saline solution are also methods that can be used to ‘salt load’.

In some cases, removing the tumor through surgery is not an option and the pheochromocytoma is managed with medications that are combined to control the effects of the excess hormones. It is important to know that pheochromocytoma has not been effectively treated with chemotherapy and radiotherapy.