Pheochromocytoma: treatment, diagnosis, causes, symptoms

What is pheochromocytoma?

Pheochromocytoma is a tumor developed mainly in the region of the adrenal glands. It can cause hypertension, it is more frequent in people between 30 and 50 years old and for every 3 patients, 1 dies before the diagnosis.

The adrenal gland, located at the back of the abdomen and above the kidneys, is responsible for producing adrenaline and norepinephrine. These hormones are responsible for the body’s response to extreme situations and relaxation. With the onset of the disease, these hormones can change.

The disease can also appear in the heart, skull, neck or chest. If this occurs, the disease is called paraganglioma or extra-adrenal pheochromocytomas (occurs in 10% of cases).

Causes of pheochromocytoma

It is not yet known what causes the disease to appear, but it is believed that it can be a hereditary problem. What is known is that there are genetic disorders, such as neurofibromatosis and von Hippel-Lindau syndrome.

The disease is linked to multiple type 2 endocrine cancer , a neoplasm that affects the adrenal and extra-adrenal glands, in addition to being a genetic disease. Tumors are caused by hyperplasia of chromaffin cells in the adrenal medulla or by extra-adrenal chromaffin tissue that caused chromaffin cell migration to fail during embryo development.

What are the symptoms of pheochromocytoma?

Symptoms vary between patients. While some may demonstrate several of them, some patients may have no symptoms at all.

The main symptoms of the disease are:

  • Headache;
  • Hypertension;
  • Palpitation
  • Shortness of breath;
  • Anxiety;
  • Nervousness;
  • Pallor;
  • Abdominal pain;
  • Hemorragia retiniana;
  • Tremors;
  • Cold;
  • Increased appetite;
  • Intense Sister;
  • Weight loss;
  • Strong or fast heartbeat.

Still, some symptoms can appear as small attacks, which last for a short time (from 15 to 20 minutes). They are:

  • Anxiety or stress;
  • Changes in body position;
  • Medicines such as decongestants and stimulants;
  • Physical effort.

Diagnosis of pheochromocytoma

The specialist doctors to treat pheochromocytoma are nephrologist, endocrinologist and general practitioner. In order for them to be able to give the diagnosis more quickly, it is interesting to advance some information, such as:

  • How long have the symptoms appeared and what are they;
  • The patient’s medical history and the medications used by the patient.

Going with this information is good, as there are some questions that the doctor can ask, such as:

  • When the symptoms started;
  • What are the symptoms;
  • What medications are used by the patient;
  • What are the activities performed that seem to worsen the symptoms;
  • If the patient has hypertension.

After the consultation, the doctor may order a series of tests, such as scintigraphy, MRI and tomography. In addition, urine and blood tests can be ordered by the doctor to make the diagnosis more accurate.

Tests known as urinary vanillmandelic acid (VMA), urine metanephrines and normetanephrines, plasma free metanephrines, plasma catecholamines, urinary free catecholamines (norepinephrine, epinephrine and dopamine) and provocative tests with glucagon and suppressants with clonidine can also be done.

Performing tests that control the amount of hormones in the adrenal glands is essential for the diagnosis of pheochromocytoma to be given.

Diagnostic evaluations can be done in three ways: laboratory evaluation, radiological evaluation and genetic evaluation.

Laboratory evaluation

Laboratory diagnosis can be assessed by measuring the catecholamines and their metabolites in urine and plasma. The clinical condition when making the laboratory diagnosis must be taken into account, since several drugs and some clinical situations lead to false positive results for pheochromocytoma.

Drugs such as tricyclic antidepressants should be discontinued for 2 weeks before hormonal assessment, if possible.

Radiological evaluation

The evaluation should be done only when biochemical tests confirm the diagnosis of catecholamine-secreting tumor. In 85% of the cases they are located in the adrenals and in the abdomen in 95% of the cases.

Magnetic resonance imaging and tomography can be used to diagnose pheochromocytoma, with a sensitivity of 95%.

Genetic evaluation

Approximately 40% of cases of pheochromocytoma and paragangliomas have an identified genetic mutation.

Treatment for pheochromocytoma

The treatment of pheochromocytoma is usually done with the removal of the tumor through surgery. The surgery performed is laparoscopy, with small incisions and because it is not very invasive. The gland is removed entirely and after that, the replacement of mineralocorticoids and glucocorticoids is done.

Before performing the operation, it is necessary to control hypertension. The medications indicated for this are:

  • Beta-blockers ( Propanolol ): inhibit the adrenaline hormone, slowing the heart down with less force and also leaving the veins more relaxed.
  • Alfablockers ( Prazosin or Doxazosin ): they are able to prevent noradrenaline from stimulating the muscles in the arteries and veins, reducing hypertension.
  • Calcium channel blockers ( Nifedipino ): they are indicated for normotensive patients with episodes of arterial hypertension. The drug is used intravenously.

Other drugs can also be used to treat the disease if those mentioned above have no effect.

If the tumor is malignant, complementation with chemotherapy or radiation therapy may be necessary. Who will say this will be the oncologist.

Attention! 

NEVER self-medicate or stop using a medication without first consulting a doctor. Only he will be able to tell which medication, dosage and duration of treatment is the most suitable for his specific case. The information contained on this site is only intended to inform, not in any way intended to replace the guidance of a specialist or serve as a recommendation for any type of treatment. Always follow the instructions on the package insert and, if symptoms persist, seek medical or pharmaceutical advice.

Complications

Complications can arise if the disease is not treated. The main complications are known as:

  • Myocardial infarction;
  • Hypertensive crises;
  • Multiple organ failure;
  • Cardiac arrhythmia.

Most tumors are benign and, with surgery, the postoperative period and the continuity of life are normal. It is believed that only 1 case in 10 has a recurrence of the tumor in the other gland.

Prevention

There are no ways to prevent pheochromocytoma disease. The indicated is to consult the specialist doctor as soon as the first symptoms appear.


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