Pheochromocytoma - Symptoms, causes and treatment

Pheochromocytoma is a benign tumor that forms in the center of the adrenal gland. This tumor interferes with the work of hormones, causing the sufferer to experience high blood pressure

Pheochromocytoma or pheochromocytoma is generally a benign tumor and only about 10% are malignant. However, if not treated properly, pheochromocytoma can lead to sustained high blood pressure that has the potential to damage the heart, brain, lungs, and kidneys.

Causes of Pheochromocytoma

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

Pheochromocytoma interferes with the work of chromaffin cells in charge of producing the hormones adrenaline and noradrenaline. In patients with pheochromocytoma, the production of these hormones is disrupted, resulting in changes in heart rate, blood pressure, and blood sugar levels.

Although very rare, pheochromocytoma can also occur outside the adrenal glands, for example in the abdominal area (paraganglioma).

Pheochromocytoma is more often experienced by people with genetic disorders that run in families, such as:

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

In addition, there are several factors that can trigger symptoms in patients with pheochromocytoma, namely:

• Exhaustion
• Stressed or anxious
• Labor
• Changes in body position
• Surgery and anesthesia
• Drug abuse, such as amphetamines and cocaine
• Consumption of foods high in tyramine (a substance that can change blood pressure), such as fermented, preserved, pickled, overcooked foods, such as cheese, beer, wine, chocolate, and smoked meats

Pheochromocytoma Symptoms

In some cases, pheochromocytoma does not cause certain symptoms. However, when a pheochromocytoma causes an increase in the production of hormones in the adrenal glands, symptoms may last from a few minutes to a few hours. These symptoms include:

• Headache
• High blood pressure
• Excessive sweat
• Heart pounding

In addition, pheochromocytoma can also cause the following symptoms:

• Pale
• Nausea and vomiting
• Constipation
• Feeling anxious
• Difficulty sleeping
• Losing weight
• Pain in stomach or chest
• Shortness of breath
• Seizure

The bigger the tumor size, the more severe the symptoms of pheochromocytoma and it will appear more often.

When to see a doctor

Hypertension is the main sign in patients with pheochromocytoma. Therefore, consult a doctor if your blood pressure is high, especially if this occurs at a young age.

If you have been taking treatment according to the doctor's recommendations but your blood pressure is still uncontrolled, discuss it with your doctor again.

Pheochromocytoma is at risk for people with genetic disorders, such as neurofibromatosis type 1, multiple endocrine type 2, or von Hippel-Lindau disease. If you suffer from this disease, check with your doctor regularly.

Pheochromocytoma Diagnosis

As a first step, the doctor will ask the patient's and family's complaints and 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. The patient will also undergo a 24-hour urine test, which is done by storing a urine sample with each urination. The blood and urine will then be examined to detect an increase in hormone levels and metabolic products.

If the lab results indicate a possible pheochochomocytoma 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 PET scan.

If the patient is confirmed to have pheochromocytoma, a genetic test will be performed to check whether the tumor is caused by a genetic disorder.

Pheochromocytoma Treatment

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

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

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

• Alpha blocking drugs

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

• Beta-blocking drugs

  These drugs work to make the heart beat more slowly and help open blood vessels and make it more open. Examples of these drugs are atenolol, propranolol, and metoprolol.

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

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

Pheochromocytoma Complications

Pheochromocytoma can cause high blood pressure. If left untreated, this condition can damage other organs of the body and lead to the following conditions:

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

Please note that 10-15% of pheochromocytoma cases can be malignant. Malignant pheochromocytoma can spread to other body tissues, such as the spleen, liver, bones, or lungs.

Pheochromocytoma Prevention

Pheochromocytoma is difficult to prevent because the cause is unknown. However, the risk of pheochromocytoma complications can be avoided by immediately seeing a doctor if you experience symptoms of this disease, especially if there are risk factors for pheochromocytoma.