Pheochromocytoma - Symptoms, causes and treatment

Pheochromocytoma or pheochromocytoma is a tumor benign which formed in the middle adrenal glands. This tumor interferes with the work of hormones, so that causing the sufferer experiences high blood pressure. 

About 90% of pheochromocytomas are benign tumors, and only about 10% are malignant. Although benign, untreated pheochromocytoma can lead to persistent high blood pressure, which has the potential to damage the heart, brain, lungs, and kidneys.

Reason Pheochromocytoma

Pheochromocytoma occurs when a tumor develops in the chromaffin cells, which are cells in the center of the adrenal glands, in either one or both adrenal glands that are above the kidneys. However, until now, the cause of the growth of these tumors is not known with certainty.

Pheochromocytoma interferes with the work of chromaffin cells, which are responsible for producing the hormones adrenaline and noradrenaline. When a person suffers from pheochromocytoma, the production of these hormones is disrupted, resulting in changes in heart rate, blood pressure, and blood sugar.

Although very rare, pheochromocytoma can also occur outside the adrenal glands, for example in the abdominal area (paraganglioma). Pheochromocytoma is more common in people who have genetic disorders that run in families, such as:

  • Multiple endocrine neoplasia type 2(MEN2)
  • Neurofibromatosis type 1
  • Paraganglioma syndrome
  • von Hippel-Lindau disease

There are several factors that can trigger symptoms in people with pheochromocytoma, including:

  • Fatigue.
  • Stress or anxiety.
  • Labor.
  • Changes in body position.
  • Surgery and anesthesia.
  • Drug abuse, such as amphetamines and cocaine.
  • High food consumption tyramine (substances that can change blood pressure), such as fermented, preserved, pickled, overcooked foods, such as cheese, beer, wine, chocolate, and bacon.

Symptom Pheochromocytoma

In some cases, the pheochromocytoma is asymptomatic. However, when a pheochromocytoma causes an increase in the production of hormones in the adrenal glands, symptoms may last from a few minutes to several hours. These symptoms include:

  • Headache
  • Heart beat
  • High blood pressure
  • Excessive sweating

In addition, pheochromocytoma can also cause symptoms such as:

  • Pale
  • Nausea and vomiting
  • Constipation
  • Feeling anxious
  • Hard to sleep
  • Weight loss
  • Pain in the stomach or chest
  • Hard to breathe
  • Seizures

The larger the size of the tumor, the symptoms of pheochromocytoma will be more severe and appear more often.

When have to go to the doctor

Hypertension is the main sign that can be found in patients with pheochromocytoma. Consult a doctor if your blood pressure is high, especially if this occurs at a young age.

If you have been taking medication as recommended by your doctor but your blood pressure is still uncontrolled, discuss it again with your doctor.

Pheochromocytoma is at risk for people with genetic disorders, such as neurofibromatosis type 1, multiple endocrine type 2, or von Hippel-Lindau disease. Patients with this disease are advised to regularly check with the doctor.

Diagnosis Pheochromocytoma

As an initial examination, the doctor will ask for complaints and ask about the patient's and family's medical history. The doctor will then perform a physical examination, including checking the patient's blood pressure.

To confirm the diagnosis, the doctor will ask the patient to undergo a blood test and a 24-hour urine test, in which the patient needs to save a urine sample with each urination. The blood and urine will be tested in a laboratory to detect increased hormone levels and metabolic products.

If the lab results suggest a possible pheochoromocytoma or paraganglioma, the doctor will ask the patient to undergo a scan to determine the location and size of the tumor. Scans can be done with an MRI, CT scan, or pocitron emission tomography (PET scan).

If the patient is confirmed to have pheochromocytoma, then genetic testing can be done to check whether the tumor is caused by a genetic disorder.

Treatment Pheochromocytoma

Surgery is the main treatment for pheochromocytoma. This action is taken to reduce excess hormone production, so that blood pressure becomes more stable.

Usually the doctor will remove the tumor or the entire adrenal gland with the laparoscopic method, which is a surgical technique with small incisions, using a special tool equipped with a camera.

For 7-10 days before surgery to remove the tumor, the doctor will give drugs to stop the work of the adrenal hormone, so that the patient's blood pressure is more stable during the operation. The drugs include:

  • Drugalpha blocker

    This drug is useful for increasing blood flow and lowering blood pressure. An example of this class of drugs is doxazosin.

  • Beta blockers

    This medicine makes the heart beat more slowly and helps blood vessels open and relax more. Examples of these drugs are atenolol, metoprolol, and propranolol.

The use of alpha and beta blocking drugs can lower blood pressure, so patients need to eat high-salt foods to prevent low blood pressure during and after surgery.

If the tumor is malignant and cannot be surgically removed, radiotherapy and chemotherapy are necessary to inhibit its growth.

Complications of Pheochromocytoma

Pheochromocytoma can cause high blood pressure. If left untreated, this condition can damage other organs of the body and result in:

  • stroke
  • Heart disease
  • Kidney failure
  • Eye nerve damage
  • Acute respiratory distress

Although rare, 10-15% of pheochromocytomas can be malignant. Malignant pheochromocytoma can spread to other body tissues, such as to the spleen, liver, bones, or lungs.

Prevention of Pheochromocytoma

This disease is difficult to prevent because the cause is unknown. However, to prevent complications from pheochromocytoma which can be fatal, immediately consult a doctor if you feel the symptoms of this disease, especially if you are at risk of suffering from pheochromocytoma.