Pheochromocytoma

Pheochromocytoma General Information

Pheochromocytoma is the generic medical term used to describe a neuroendocrine tumor that has evolved in the medulla of the adrenal glands. It originates in the chromaffin cells, and is a remnant of chromaffin tissue. Normally this tissue involutes after birth, however in such cases the involution did not occur resulting in the appearance of the tumor. It secretes catecholamine in excessive quantities (typically epinephrine and norepinephrine, but other substances can be secreted as well).

Due to a series of coincidences, the Pheochromocytoma is also colloquially known as “the 10% tumor”. The tumor is malignant in 10% of the cases, while in 10% of the cases the tumor appears in childhood and 10% of the tumors are genetically transmitted in the family. 10% of the tumors are situated around the adrenal glands, in the chromaffin tissue; 10% of the patients also present bilateral disease, and 10% of the tumors recur after resection.

This type of tumor is part of the multiple endocrine neoplasia syndrome, along with thyroid cancer and parathyroid adenomas, and they are caused by the mutation of a particular gene, the RET oncogene. If discovered in time, the affection is possible to be cured; however the consequences for treatment not being applied in time or at all may be extremely severe.

Pheochromocytoma Symptoms

Many of the symptoms that derive from the presence of a Pheochromocytoma within the organism are similar to those caused by hyperactivity of the sympathetic nervous system. Such symptoms that may be experienced by a patient with such neoplasm may include, but not limit to palpitations, heart rate increase and elevation of blood pressure. Diaphoresis, headaches, pallor and weight loss, often coupled with acute anxiety have been noted in such cases, and microscopic studies may reveal the presence of localized amyloid deposits. The catecholamine secreted by the tumor stimulates the process of lipolysis eventually leading to increased blood glucose levels. It has been noted that the presence of a Pheochromocytoma in the organism can cause hypertension. In most cases where present, this is particularly difficult to treat as it does not seem to respond to common therapies, and it may aggravate into malignant hypertension or excessively high blood pressure, which may prove fatal for the patient.

This is not a complete list of all the possible symptoms that are associated with the presence of a Pheochromocytoma in the organism. In other words, if you are suffering from this condition, you may experience a set of reactions that are not listed here, since every organism tends to respond to different affections in different and sometimes unique ways. We strongly advise all patients who start experiencing any unusual, uncommon or disturbing symptoms to immediately contact their personal health care specialist or to go to the nearest hospital in order to seek medical attention.

Pheochromocytoma Treatment

The treatment of choice in curing patients with Pheochromocytoma is surgery. The tumor needs to be removed from the body, and several procedures are available allowing the specialized health care professionals to pick from a variety of options. The most commonly employed procedures are the resection of the tumor by means of laparoscopic operation, or by performing an open laparotomy. However, since the operations may prove extremely difficult to perform, and as there is a risk for severe complications to occur, it is highly advised that such operations are carried out only in specialized centers which have extensive experience in the field. Such centers should also provide all the necessary additional resources, such as appropriate anesthesia and endocrine. The specialized health care professionals may also find it necessary to completely remove the affected adrenal gland by performing an adrenalectomy.

In order to minimize the risks of any case of severe hypertension occurring during the surgical operation, the patient may be administered a treatment with Phenoxybenzamine prior to the operation. This drug is an irreversible alpha blocker of the adrenoreceptors, and prevents the blood pressure from rising to dangerous levels during the removal of the tumor. Alternative practices involve administering of a combined alpha/beta blocker (such as Labetalol) that slows the heart rate during the surgery. However, it is highly advised against administration of pure beta blockers (e.g. Atenolol) while the Pheochromocytoma is still inside the organism, as this may lead to severe complications and potentially life threatening hypertension.

In the vast majority of cases the patient bearing the Pheochromocytoma is affected by an almost complete inhibition of the renin-angiotensin activity, leading to severe volume depletion. After the Pheochromocytoma is removed, thus removing the main source of catecholamines, the organism is confronted both with volume depletion as well as with a reduced activity of the sympathetic system, situation which may lead to severe hypotension. As a counter to that, before the patient goes into surgery he or she is administered salt – either through dietary methods, by adopting a salt-rich diet before the operation, or through salt administration, either through direct salt replacement or through an intravenous drip of saline solution.

There are other methods available for treating this affection, that are not listed here. In order to obtain further information regarding the specific procedures that may be employed in treating this affection, it is recommended that you consult a specialized health care professional as he or she may be able to provide you with additional information.

If the symptoms that you may experience lead to the conclusion that you may have a Pheochromocytoma, it is best that you consult your personal health care specialist as soon as possible. Early diagnosis of the condition may further assist the various therapies by reducing the recovery time, or simply by not giving the affection enough time to evolve into more complex forms. The specialized health care professional may require additional testing in order to determine the exact nature of the affection and may ask you to undergo some generic tests, as well as a complete medical examination. Once diagnosed, the consulting physician will prescribe the treatment best suited to your case, based on your age, medical history and general health condition. Feel free to discuss the treatment, its risks and benefits with the prescribing doctor in order to find out exactly why you have been prescribed a certain therapy and to obtain additional information.