Pheochromocytoma: Risk Factors, Causes, Symptoms, Diagnosis and Treatment

What is it? It is a rare tumor located in the adrenal glands , which are located above the kidneys.

The adrenal glands produce several hormones, such as aldosterone, testosterone, cortisol, norepinephrine and epinephrine, which are responsible for regulating vital bodily functions such as blood pressure, stress, blood sugar, immune response, metabolism and the heart rate.

Pheochromocytoma is rarely cancerous, but it can be dangerous due to the fluctuation of hormone levels.

Risk factor’s

It is believed that pheochromocytoma may be linked to conditions in which hypoxia (reduced oxygen supply) occurs. These include:

  • Severe hypertension (high blood pressure).
  • Congenital heart disease.
  • This pathology can develop at any age, but it is more common from early to mid-adulthood.

Causes of pheochromocytoma

Recent research indicates that it can be hereditary. That means that it is transmitted from parents to children. Patients who inherit pheochromocytoma from their parents may also suffer from associated genetic conditions. These conditions include:

  • Von Hippel-Lindau disease (VHL) . It is a condition in which cysts and tumors grow in the central nervous system, the brain, the kidneys, the adrenal glands, or other areas of the body.
  • Neurofibromatosis 1 (NF1). The development of tumors in the skin and the optic nerve.
  • Multiple endocrine neoplasia type II (NEM). It is a form of thyroid cancer that develops in conjunction with pheochromocytoma.
  • Pheochromocytoma may be associated with congenital heart disease. Heart disease can cause hypoxia that contributes to this disease.

The symptoms

They are the result of the production of excess hormones and the release of them. A sudden large increase in adrenal hormones is called the adrenergic crisis (AC), which causes hypertension and tachycardia (rapid heart rate).

The most common symptoms are:

  • Episodic and sudden onset of severe headaches.
  • Perspiration.
  • Abdominal pain.
  • Hypertension (high blood pressure) that may be resistant to conventional medications.
  • Rapid heart rate
  • Irritability and anxiety

Diagnosis

The diagnosis has improved with modern technology. However, it is still difficult to do it. If you have symptoms, the doctor can order tests. These include

  • Imaging tests to look for tumors.
  • Magnetic resonance images .
  • Laboratory tests to evaluate hormone levels.
  • Blood plasma (test of catecholamine and metanephrine levels).
  • Urine test (tests of catecholamine and metanephrine levels that may have been missed in the blood test)

Treatment for pheochromocytoma

The best treatment is surgery, despite being a delicate procedure. Without extensive patient preparation, it can be life-threatening.

Doctors prepare the patient through:

  • Discuss all existing medications, as some may interfere with pheochromocytoma during surgery.
  • Patients after surgery may have short-term problems with the regulation of adrenal hormone.
  • The steroids can substitute the proper functioning of these glands, until this normal function restarts.