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Risk Factors, Causes and Symptoms

Your body has two adrenal glands, one located above each of your kidneys. The adrenals produce hormones that give the organs and tissues in your body instructions on how they should function.

These hormones regulate vital bodily functions such as:

Pheochromocytoma (PCC) is a rare tumor that can form in cells in the middle of the adrenal glands. The tumor can cause the adrenal glands to make too much of the hormones norepinephrine (noradrenaline) and epinephrine (adrenaline).

Together these hormones control heart rate, metabolism, blood pressure, and the body’s stress response. Increased levels of these hormones can put the body into a stress-response state, causing blood pressure to increase.

The cells that make up these tumors are known as chromaffin cells. When chromaffin cells grow abnormally within the adrenal glands they are called pheochromocytomas, but when they develop elsewhere in the body they are called paragangliomas.

Both PCCs and paragangliomas can produce excessive hormones called catecholamines.

A PCC can develop at any age, but is most common in early to middle adulthood. It’s believed that the condition is often genetically inherited.

People who inherit PCC from their parents may also develop associated genetic conditions. These conditions include:

  • Von Hippel-Lindau disease, a condition where cysts and tumors grow in the central nervous system, kidneys, adrenal glands, or other areas of the body, most are non-cancerous
  • Neurofibromatosis type 1, the development of tumors on the skin and optic nerves
  • Multiple endocrine neoplasia type 2 (MEN2), a form of thyroid cancer that develops in conjunction with PCC

A sudden large increase in adrenal hormones is called an adrenergic crisis (AC). AC causes severe high blood pressure (hypertension) and a rapid heart rate (tachycardia).

Common symptoms of PCC are:

  • headaches
  • sweating
  • high blood pressure that may be resistant to conventional medications
  • rapid heart rate or palpitations
  • abdominal pain
  • irritability and anxiety
  • constipation

Common risk factors for AC are:

The presence of the first four symptoms listed above is highly suggestive of PCC. Without the presence of these four symptoms, the diagnosis can actually be excluded. The diagnosis of PCC has improved with modern technology.

There are several different tests your doctor may use to diagnose PSS. These include:

Surgical removal of the tumor is usually the main method of treatment. However, because of the importance of the adrenal glands, this surgery can be very difficult.

If a PCC is found to be cancerous, cancer treatments such as chemotherapy and radiation therapy may be required after surgery.

After surgery you may have short-term problems with low blood pressure or low blood sugar.

Without treatment, those with a PCC are at a higher risk for the following conditions:

However, as with any surgery, treating PCC surgically may involve complications. The surgery affects powerful hormones in the body. During surgery, some of the conditions that may develop include:

  • high blood pressure crisis
  • low blood pressure crisis
  • irregular heartbeat

In rare cases, PCC may be cancerous. In these cases, surgery is followed with radiation therapy or chemotherapy.

The outlook for a person with a PCC depends on whether the tumor is cancerous. People who had a PCC that wasn’t cancerous had a 5-year survival rate of 96 percent. The survival rate was 44 percent for people who had a cancerous tumor.

Early diagnosis isn’t always enough to ensure successful treatment. Because of the difficulty of the surgery, seek out a surgeon who’s highly skilled and able to handle possible complications.

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