Pheochromocytoma: Causes, Symptoms, Treatment, Prognosis
What is Pheochromocytoma?
Pheochromocytoma is an extremely rare pathological condition of the adrenal glands where there is development of benign tumors on the cells present in the center of the gland causing symptoms. The adrenal glands are located at the top of each kidney and are responsible for producing hormones which provide instructions to all the organs and tissues of the body.
Pheochromocytoma, is a condition in which because of the benign tumors, start producing hormones which result in elevation of blood pressure persistently and if this condition remains untreated it may cause severe damage to other vital organs of the body.
Pheochromocytoma can develop at any age but is mostly seen in individuals between the age range of 20-50. Once Pheochromocytoma is diagnosed, the best way to treat it is to remove the benign tumors surgically. Once the tumors are removed in majority of the cases the blood pressure returns back to normal.
What are the Causes of Pheochromocytoma?
There is no clear cut reason as to what causes development of Pheochromocytoma. Pheochromocytoma normally develops in the specialized cells of the adrenal gland called the chromaffin cells. These cells are located right in the center of the adrenal gland and are responsible for secreting hormones primarily adrenaline. The hormone adrenaline comes to play when an individual is under stress and calms the body down. This hormone results in an individual having higher blood pressure readings and a fast heart rate. Pheochromocytoma causes irregular and excessive release of adrenaline.
What are the Risk Factors for Pheochromocytoma?
There are certain rare congenital conditions that put an individual at risk for developing Pheochromocytoma. These medical conditions are:
Multiple Endocrine Neoplasia - Type II: This is a disorder in which there is development of tumors in the endocrine system of the body including the thyroid, parathyroid, lips, tongue and the gastrointestinal tract.
Von Hippel-Lindau Disease: This disease can result in development of tumor in the central nervous system, endocrine system, pancreas, and kidneys.
Neurofibromatosis-1: This condition results in development of tumor in the skin and the optic nerve.
What are the Symptoms of Pheochromocytoma?
Some of the symptoms of Pheochromocytoma are:
- Persistent high blood pressure is one of the common symptoms of Pheochromocytoma
- Persistent rapid heartbeat
- Excessive sweating
- Severe persistent headaches can also be a symptoms of Pheochromocytoma.
- Having a pale face
- Shortness of breath.
Some of the less common symptoms of Pheochromocytoma are:
- Severe anxiety
- Abdominal pain
- Unintentional weight loss.
The individual suffering from symptoms of Pheochromocytoma will experience symptoms in episodic bursts which will last from 15-20 minutes. There may be multiple spells during a 24 hour period. In between the episodes the blood pressure of the individual may or may not return back to normal.
How is Pheochromocytoma Diagnosed?
If Pheochromocytoma is suspected, then the doctor will order a series of test to confirm the diagnosis. These tests are:
Laboratory Tests: This will include checking the levels of adrenaline in the body:
Urinalysis: A urinalysis will also be performed to check for any signs pointing towards a Pheochromocytoma.
Blood Test: A CBC will also be done to rule out infection as a cause for the symptoms.
Radiological Studies: The doctor will also order radiological studies in the form of CT or MRI of the abdomen and pelvis region if Pheochromocytoma is suspected on laboratory tests. The CT scan or the MRI will clearly show presence of tumors in the adrenal gland. A biopsy of the tumor may also be done to check whether the tumor is benign or malignant.
These above tests virtually confirm the diagnosis of Pheochromocytoma.
What is the Treatment for Pheochromocytoma?
As stated above, the best possible treatment for Pheochromocytoma is removal of the tumors surgically. Before the patient undergoes surgery for Pheochromocytoma, the patient's blood pressure is brought under control with the help of medications so as to lower the risk of any intraoperative or perioperative complications.
For bringing the blood pressure back to normal, basically two forms of medications will be used. The first class of medications is the Alpha blockers. These medications prevent stimulation of the muscles in the walls of the arteries thus keeping them open so that blood can flow through them easily and thus normalizing the blood pressure. Some of the medications of this class are: Cardura, Minipress, and Hytrin.
The second class of medications that may be used to bring down the blood pressure back to normal levels are Beta Blockers which cause the heart to beat slowly and also keep the vessels and arteries open so that blood can flow through them easily and normalize the blood pressure. Some of the examples of this class of medications are: Atenolol, metoprolol, and propranolol. These medications may be given for a period of a week to 10 days. Once the blood pressure has normalized then the patient is prepared fur surgery to remove the tumors and treat Pheochromocytoma.
For treating Pheochromocytoma, the surgery may involve removal of the part of the adrenal gland containing the Pheochromocytoma in most of the cases. The remaining part of the adrenal gland carries out it functions normally. This is usually done laparoscopically and is a minimally invasive procedure. After successful completion of the surgery and removal of the Pheochromocytoma the blood pressure in majority of the cases returns back to normal. In some cases where the adrenal gland has already been removed and there is still presence of a tumor then in such cases only the tumor will be removed in its entirety.
Malignant tumors is extremely rare in Pheochromocytoma but if present then standard procedure that are followed for cancer treatment needs to be followed to include surgical removal of the tumor followed by chemo and radiation therapy for treatment of malignant tumors from Pheochromocytoma.
What is the Prognosis of Pheochromocytoma?
The prognosis of Pheochromocytoma is excellent given that there are no intraoperative or perioperative complications due to high blood pressure. Coming to the recurrence rate, there are about 10% chances that even after a successful surgery there may be a recurrence of Pheochromocytoma.