Pheochromocytoma

Pheochromocytoma is a rare neuroendocrine tumor that arises from adrenal glands, or extra-adrenal chromaffin tissue that failed to involute after birth. The tumor produces extra adrenaline – a hormone which affects the following functions of your body: blood pressure, heart rate, digestion, breathing, sexual arousal, urination, and sweating. Moreover, adrenaline helps the body react and respond to stressful situations by boosting the supply of glucose and oxygen to the muscle and brain. Pheochromocytoma secretes extra adrenaline in an uncontrolled way and can cause severe health problems, including heart attack, stroke, and even death.

Pheochromocytoma Drugs

Pheochromocytoma Symptoms

Individuals with a Pheochromocytoma typically have four classic symptoms, like sweating, headache, heart palpitations, and high blood pressure (hypertension). However, some people (up to 10%) never have symptoms of a pheochromocytoma and discover the disease incidentally. In some cases, the high blood pressure is a nonpermanent symptom. Sometimes, patients with Pheochromocytoma may have spells with the following symptoms:

  • Anxiety, sense of doom
  • Constipation
  • Epigastric pain
  • Flank pain
  • Nausea
  • Tremor
  • Weakness

Other symptoms associated with Pheochromocytoma may also occur:

  • Cafe au lait spots
  • Cardiomyopathy
  • Fever
  • Hypertensive retinopathy
  • Ileus
  • Neurofibromas
  • Pallor
  • Postural hypotension:
  • Pulmonary edema
  • Tachyarrhythmias
  • Tremor
  • Weight loss

The frequency of spells may vary in time (from monthly to several times per day), duration (from seconds to hours) and intensity (become more severe with the tumor growth).

Pheochromocytoma Causes

It is not know exactly what causes Pheochromocytoma. The tumor develops in chromaffin cells, which are located in the adrenal gland center, usually in the center of one or both adrenal glands. In some cases, the tumor may occur outside of glands. Pheochromocytoma may be caused by overactivity of the sympathetic nervous system, which governs the release of a hormone in response to fear or stress. This condition may be caused by the following factors:

  • Excessive use of certain drugs
  • Withdrawal from drugs (e.g., when you suddenly stop blood pressure medicines)
  • Panic attacks
  • Spinal cord injuries

Pheochromocytoma may develop at any age, but they mostly occur from early to mid-adulthood. It is also believed that Pheochromocytoma may be inherited, i.e. passed down through families. Patients who inherit this type of tumor may also have the following genetic conditions:

  • Multiple endocrine neoplasia type II (MEN)
  • Neurofibromatosis 1 (NF1)
  • Von Hippel-Lindau disease (VHL)

Pheochromocytoma Diagnosis

Pheochromocytoma may be diagnosed in anyone with the following symptoms: headache, heart palpitations, and sweating. If a patient has no habits or risk factors that may cause these symptoms, a doctor may prescribe certain tests to measure the culprit hormones metabolites. Pheochromocytoma is diagnosed by the following ways:

  • Plasma metanephrine testing: 85% specificity, 96% sensitivity
  • 24-hour urinary collection for metanephrines and catecholamines: 99.7% specificity, 87.5% sensitivity

After biochemical studies have confirmed the diagnosis of Pheochromocytoma, the following imaging studies should be made:

  • Abdominal CT scanning
  • MRI
  • Scintigraphy
  • PET scanning

There may be additional tests to study a familial syndrome in patients with confirmed pheochromocytoma:

  • Simultaneous serum calcium level and serum intact parathyroid hormone level
  • Screening for mutations
  • Genetic testing for mutations
  • Consultation with an ophthalmologist

Pheochromocytoma Treatment

There is no approved medication or vaccine to treat Ebola. However, symptoms of Ebola may be treated as soon as they appear. When the treatment is used early, the patient’s chances of survival may significantly raise. The following interventions may help to overcome Ebola:

  • Balance electrolytes and provide intravenous fluids
  • Maintain blood pressure and oxygen status.
  • Treat other infections once they occur.

A patient’s ability to recovery from Ebola depends on their immune response and good supportive care. After patients recover from Ebola infection, they develop antibodies that last for 10 years or longer. However, they may develop long-term post recovery complications, like vision and joint problems.