Description

A pheochromocytoma (fee-or-kroe-moe-sy-TOE-muh) is a rare tumor that grows in the adrenal gland. Most often, the tumor is not cancer. When the tumor is not cancer is called benign.

You have two adrenal glands — one on the top of each kidney. The adrenal glands produce hormones that help control the key processes in the body, such as blood pressure. Usually, a pheochromocytoma is formed in the adrenal gland. But the tumors can grow in both adrenal glands.

With a pheochromocytoma, a tumor secretes hormones that can cause a variety of symptoms. These include high blood pressure, headache, sweating, and symptoms of a panic attack. If a pheochromocytoma is not treated, serious or life-threatening damage to other systems of the body that can happen.

Surgery to remove a pheochromocytoma often becomes blood pressure in a healthy range.

Symptoms

A pheochromocytoma often the cause of the following symptoms:

  • The high blood pressure.
  • The pain of a headache.
  • Excessive sweating.
  • A fast heartbeat.

Some people with pheochromocytomas also have symptoms such as:

  • Nervous, trembling.
  • The skin becomes of a lighter color, also called pale.
  • Shortness of breath.
  • Panic attack-type symptoms, which can include sudden onset of intense fear.
  • Anxiety or a sense of doom.
  • Vision problems.
  • The constipation.
  • The loss of weight.

Some people with pheochromocytomas do not have symptoms. They do not realize that they have the tumor until an imaging test that happens to find it.

Symptom spells

Most often, the symptoms of pheochromocytoma come and go. When they start suddenly and keep coming back, they are known as spells or attacks. These spells may or may not have a trigger that you can find.

Certain activities or conditions that can lead to a spell, such as:

  • Hard physical work.
  • Anxiety or stress.
  • Changes in body position, such as bending, or sitting or lying down to standing.
  • Labor and delivery.
  • The surgery and the medicine that causes you to be in a state similar to sleep during the surgery, it is called an anesthetic.

Foods rich in tyramine, a substance that affects blood pressure, can also trigger spells. Tyramine is common in foods that are fermented, age, vinegar, curing, in excess or in a poor state. These foods include:

  • Some cheeses.
  • Some beers and wines.
  • Soybeans and products made from soy.
  • Chocolate.
  • Dried or smoked.

Certain medications and drugs that can trigger spells include:

  • Medicines for depression called tricyclic antidepressants. Some examples of tricyclic antidepressants include amitriptyline and desipramine (Norpramin).
  • Medicines for depression called monoamine oxidase inhibitors (Maois) such as phenelzine (Nardil), tranylcypromine (Parnate), and isocarboxazid (Marplan). The risk of spells is even greater if these medicines are taken with food or beverages high in tyramine.
  • Stimulants such as caffeine, amphetamines, or cocaine.

When to see a doctor

High blood pressure is one of the main symptoms of a pheochromocytoma. But most of the people who have high blood pressure do not have a tumor in the adrenal glands. Talk with your health care professional if any of these factors apply to you:

  • The spells of the symptoms associated with pheochromocytoma, such as headaches, sweating, and a rapid pounding heartbeat.
  • Difficulty to control the high blood pressure with your current treatment.
  • The high blood pressure that started before 20 years of age.
  • Recurrent large increases in blood pressure.
  • A family history of pheochromocytoma.
  • A family history of a genetic-related condition. These include multiple endocrine neoplasia type 2 (MEN 2), von Hippel-Lindau disease, inherited paraganglioma syndromes and neurofibromatosis 1.

Causes

The researchers do not know exactly what causes a pheochromocytoma. The tumor is formed in the cells called chromaffin cells. These cells are located in the center of the adrenal gland. That the release of certain hormones, mainly adrenaline and noradrenaline. These hormones help to control many functions of the body, such as heart rate, blood pressure and blood sugar.

Adrenaline and noradrenaline activate the body's fight-or-flight response. That response occurs when the body thinks that it is not a threat. The hormones cause the blood pressure to rise and the heart to beat faster. Also prepared in other systems of the body so that you can react quickly. Pheochromocytoma does more of these hormones are released. And this causes them to be released when you're not in a situation of threat.

The majority of chromaffin cells are produced by the adrenal glands. But small groups of these cells are also in the heart, the head, the neck of the bladder, in the area of the stomach and along the spine. The chromaffin cell tumors located outside the adrenal glands are referred to as paragangliomas. Can cause the same effects in the body as a pheochromocytoma.

Risk factors

A person's age, and certain medical conditions can increase the risk of a pheochromocytoma.

The majority of pheochromocytomas are found in people between the ages of 20 and 50. But the tumor can form in any age.

People who have certain rare genetic conditions have a greater risk of pheochromocytomas. Tumors can be benign, which means they are not cancer. Or they can be malignant, which means they are cancer. Often, benign tumors associated with these genetic conditions form in both adrenal glands. Genetic conditions associated with pheochromocytoma include:

  • Multiple endocrine Neoplasia type 2 (MEN 2). This condition can cause tumors in more than one part of the body of the hormone system of development, called the endocrine system. There are two types of MEN 2 — type 2A and type 2B. Both can involve pheochromocytomas. Other tumors associated with this disease can appear in other parts of the body. These parts of the body including the thyroid, the parathyroid glands, the lips, the mouth and the digestive system.
  • The Von Hippel-Lindau disease. This condition can cause tumors in many parts of the body. Possible sites include the brain and the spinal cord, the endocrine system, the pancreas and the kidneys.
  • The Neurofibromatosis 1. This condition causes tumors in the skin called neurofibromas. It can also cause tumors of the nerves in the back of the eye that connects with the brain, called the optic nerve.
  • Hereditary paraganglioma syndromes. These conditions are transmitted in families. They can result in pheochromocytomas or paragangliomas.

Complications

A pheochromocytoma can lead to other health problems. High blood pressure linked with a pheochromocytoma may cause damage to organs, especially the tissues of the heart and the blood vessels of the system, the brain and the kidneys. This damage can lead to dangerous conditions, including:

  • Diseases of the heart.
  • Stroke.
  • The renal failure.
  • The loss of vision.

Cancerous tumors

Rarely, a pheochromocytoma is a cancer, and the cancer cells spread to other parts of the body. The cancer cells of pheochromocytoma or paraganglioma more often travel in the lymphatic system, bones, liver or lungs.

Diagnosis

To find out if you have a pheochromocytoma, your health practitioner will likely order a number of tests.

Laboratory tests

These tests measure the levels of the hormones adrenaline and noradrenaline, which are substances that may come from those hormones called metanephrines. The elevated levels of metanephrines are more common when a person has a pheochromocytoma. Metanephrine levels are less likely to be high when a person has symptoms due to something other than pheochromocytoma.

  • 24-hour urine test. In this test, you collect a sample of urine each time you urinate more than 24 hours. Ask for written instructions on how to store, tag and return of the samples.
  • Blood test. A health care professional will take a blood sample to be tested in the laboratory.

For both types of tests, ask your health professional if you need to do anything to prepare. For example, you may be asked not to eat for a certain amount of time before the test. This is called fasting. Or you may ask for you to stop taking a certain medicine. Do not skip a dose of this medication unless a member of your health care team tells you and gives you instructions.

Imaging tests

If the results of laboratory tests to find the signs of a pheochromocytoma, the imaging tests are necessary. Your health practitioner will likely order one or more of these tests to find out if you have a tumor. These tests may include:

  • CTscan, which combines a series of X-ray images taken from different angles around your body.
  • Magnetic resonance imaging, which uses radio waves and a magnetic field to produce detailed images.
  • M-iodobenzylguanidine (MIBG) imaging, an analysis that can detect tiny amounts of the injection of a radioactive compound. The compound is absorbed by pheochromocytomas.
  • Positron emission tomography (PET), an analysis can also detect radioactive compounds taken up by a tumor.

A tumor in the adrenal gland can be found during imaging studies performed for other reasons. If that happens, the health professionals often order more tests to determine if the tumor needs to be treated.

Genetic testing

Your health care professional may recommend genetic testing to see if a pheochromocytoma is associated with a genetic condition. The information about the possible genetic factors may be important for many reasons:

  • Some genetic conditions can cause more of a medical problem. Thus, the results suggest the need to rule out other medical conditions.
  • Some genetic diseases are more likely to happen again or be cancer. Thus, the results of the test can affect treatment decisions or long-term plans for the monitoring of their health.
  • The results of the tests may suggest that the other members of the family should be screened for pheochromocytoma or related conditions.

Genetic counseling can help you understand the results of your genetic test. You can also help your family to manage a mental health problem linked to the stress of genetic testing.

Treatment

The main treatment for pheochromocytoma is surgery to remove the tumor. Before the surgery, your healthcare provider will likely prescribe certain medications for blood pressure. These drugs block of high adrenaline, hormones to reduce the risk of dangerously high blood pressure during surgery.

Preparation before surgery

It is likely that you will have to take medication for 7 to 14 days before surgery to help reduce the blood pressure. These medications will replace or be added to other blood pressure medicines that you take. They can also eat a high-sodium content of the diet.

Medications such as alpha blockers, beta blockers and calcium channel blockers to keep small veins and arteries open and relaxed. This improves blood circulation and lowers blood pressure. Some of these medicines can also cause the heart to beat more slowly and with less force. This can reduce the blood pressure more.

Due to these medications that can dilate the blood vessels, which causes the amount of fluid within the blood vessels to be low. This can cause blood pressure drops upon standing. A high sodium diet can draw more fluid into the blood vessels. This helps to prevent low blood pressure during and after surgery.

Surgery

More often, a surgeon makes a few small cuts, called incisions in the stomach area. Wandlike device equipped with video cameras and small tools that are placed through the courts to do the surgery. This is called laparoscopic surgery. Some surgeons do the procedure with the robotic technology. Sit on a nearby console and the control of robotic arms, which have a camera and surgical tools. If the tumor is very large, a surgery that involves a larger incision and the opening of the abdominal cavity may be necessary.

Often, the surgeon removes the entire adrenal gland that has the pheochromocytoma. But the surgeon may remove only the tumor, leaving some of the healthy tissue of the adrenal gland. This can be done when the other adrenal gland has also been removed. Or can you do when there are tumors in both adrenal glands.

If a tumor is cancer, and the cancer has spread to other organs, surgery may not be able to remove all of the cancer tissue. Remove as much of the tumor as possible along with the medical therapy could alleviate the symptoms of pheochromocytoma. It also makes the blood pressure more easy to control.

After the surgery

If a healthy one adrenal gland is maintained, it can carry out all the functions usually performed by two glands. The blood pressure usually returns to a healthy range after surgery. You will need regular check-ups with your health care professional for the rest of your life. These quotes help track your health, find other health concerns and check to see if the tumor has returned. If both adrenal glands are removed, you will have to take steroids for the rest of your life. These medications replace certain hormones that the adrenal glands do.

Treatments for cancer

Very few of the pheochromocytomas are cancer. Because of this, the research about the best treatments is limited. Treatments for cancerous tumors and cancer that has spread in the body, associated with pheochromocytoma, may include:

  • Targeted therapies. These use a drug combined with a radioactive substance that you in search of cancer cells and kills them.
  • Chemotherapy. This treatment uses powerful drugs that kill rapidly growing cancer cells. Can help relieve symptoms in people with pheochromocytomas whose cancer has spread.
  • The radiation therapy. This treatment uses beams of intense energy to kill cancer cells. It can relieve the symptoms of the tumors that have spread to the bones and cause pain.
  • The ablation. This treatment can destroy cancer tumors with freezing temperatures, high-energy radio waves or ethanol alcohol.

Preparing for your appointment

It is likely to start by seeing your primary healthcare professional. Then you may be referred to a specialist in hormone conditions that is called an endocrinologist.

Here's some information to help you prepare for your appointment. Have a family member or friend if you can. This person can help you remember the information your care team gives you.

What you can do

When you make the appointment, ask if there is something that you need to do beforehand. For example, you may be asked not to eat for a certain amount of time before the appointment. This is called fasting. Or you may need to make the medicine of changes before undergoing a specific test.

Before your appointment, make a list of:

  • Its symptoms, including changes to how you normally feel, when it started and how long they last.
  • Key personal information, including major stresses, recent life changes and family medical history.
  • All the drugs, vitamins, herbs and other supplements that you are taking, including dosage.
  • Questions to ask your health care professional.

Questions to ask your health care professional may include:

  • What is likely causing my symptoms?
  • Other that the most likely cause, what are other possible causes of the symptoms?
  • What tests do I need?
  • Is my condition likely to be short-term or ongoing?
  • What treatment do you recommend it?
  • There are other treatment options apart from the main that I have suggested?
  • I have other health conditions. How can I best manage them together?
  • There are restrictions that must be followed?
  • You should see a specialist?
  • Are there brochures or other printed material I can have? What sites do you recommend?

Feel free to ask questions during your appointment.

What to expect from your doctor

Your healthcare provider is likely to ask questions such as:

  • The symptoms been constant or come and go?
  • Nothing seems to improve the symptoms?
  • What, in any case, it seems to trigger your symptoms or worse?
  • Do you have any other medical condition? If so, what type of treatment they are receiving?
  • Do you have a family history of adrenal tumors or other tumors in the endocrine?
Symptoms and treatment of Pheochromocytoma