Pheochromocytoma

What is a pheochromocytoma?

Pheochromocytoma is a rare type of tumor. It has an adrenal gland (most often) or extrarenal localization. The adrenal glands are small paired endocrine glands located above the top of the kidneys.

Pheochromocytoma produces an excessive amount of a hormone called "adrenaline". It's a fight-or-flight hormone. The production of large amounts of this hormone causes attacks of high blood pressure, increased heart rate, sweating and headaches.

Pheochromocytoma usually occurs in adults. But it is sometimes found in children, as it can be of a family nature. People with rare diseases such as multiple endocrine neoplasia and Hippel-Lindau disease are more likely to have a pheochromocytoma.

Pheochromocytoma will be more malignant in children than in adults. But it can cause complications, even if there is no cancer. The "fight or flight" hormone that is produced leads to heart damage and even death.



What are the symptoms of pheochromocytoma?

The symptoms are different for each person. Adults and children have different symptoms.

Common symptoms include:

1) headaches – they can be mild or severe, be long or short-term;

2) sweating;

3) increased heart rate,

4) increased blood pressure, hypertensive crisis;

5) tremor (trembling of the hands and fingers);

6) pallor of the skin;

7) general weakness;

8) feelings of fear or anxiety;

9) abdominal pain or palpable swelling – this is more likely in children.

Some people with pheochromocytoma do not have any symptoms.

Most people with pheochromocytoma have very high blood pressure, which can be dangerous. Blood pressure rises or decreases if a person changes their body position or for some other reason.

Do I need to be examined? - yes. After talking with you, your doctor will prescribe an examination, which includes laboratory tests to measure the level of hormones in your body.  These include:

  • blood test,
  • urinalysis.

Before you take the tests, tell your doctor about all the medications (prescription and non-prescription) that you are taking. You may need to stop taking them before you get tested, as some medications alter hormone levels and this can make the test results incorrect.

If blood or urine tests show the presence of a pheochromocytoma, doctors prescribe examination methods to find it in the body.

These include:

  • Ultrasound of the abdominal cavity and retroperitoneal organs;
  • CT or MRI of the adrenal glands;
  • other instrumental methods, if CT or MRI cannot show a tumor in the adrenal glands.


How is pheochromocytoma treated?

 

The pheochromocytoma is removed surgically.

Before the operation, the doctor corrects blood pressure and heart rate with the help of drugs. This helps to prevent serious problems during the operation.

Medications should be taken for 2 weeks before surgery.

Pheochromocytoma is usually not malignant. But if this happens, the doctors do everything possible to cure you. They use radiation therapy, chemotherapy, and other treatments.

The main method of treatment is surgical. But pheochromocytoma can "recede" even after surgical treatment. Sometimes a primary non-malignant pheochromocytoma can become malignant. Routine post-treatment check-ups are performed as often as recommended by the attending physician.

 

What if I'm pregnant?

If you are pregnant, the doctor may prescribe symptomatic treatment for pheochromocytoma. You can be operated on if you have a period of less than 24 weeks. If the term is more than 24 weeks, it is recommended Caesarean section. With a Caesarean section, you can immediately remove the tumor. If you have a pheochromocytoma and have a history of Cesarean section, then delivery is performed through the natural birth canal.

GBUZ Moscow Clinical Scientific Center named after Loginov MHD