Causes and symptoms of pheochromocytoma, diagnosis and treatment methods
Patients diagnosed with pheochromocytoma look normal, so it is difficult to detect the disease. If a person's blood pressure rises sharply and injection pills do not help, the doctor may determine this as symptoms of pheochromocytoma.
Types and causes of pheochromocytoma
The tumor grows from the tissues of the cerebral layer of the adrenal glands and sympathetic nerve tissue nodes. According to the location, there are two types - adrenal pheochromocytoma and non-adrenal, called paraganglioma.
The pathology is the cause of many cases of symptomatic hypertension, which often disappears after the tumor is removed.
The causes of the disease are not known for certain. The tumor can be caused by a genetic defect (up to 10% of cases are familial).
Symptoms of pheochromocytoma
The tumor produces huge amounts of catecholamines, among which norepinephrine is the most abundant and adrenaline is somewhat less. The symptoms of the disease are caused by the action of excessive amounts of these hormones. The result of their production is a sympatho-adrenal crisis, which occurs in most patients with persistent hypertension. Less commonly, hypertension is absent in a hypertensive crisis with pheochromocytoma.
Crises in patients occur suddenly and are often provoked by physical activity or other factors that cause displacement of the abdominal organs, as well as hypothermia. Stress and psychological factors do not provoke a hypertensive crisis in pheochromocytoma.
Manifestations of the crisis
- headache
- profuse sweating;
- increased heart rate;
- acute pain in the chest and abdomen;
- nausea and vomiting;
- diarrhea or, conversely, constipation;
- rise in blood pressure to critical levels - over 250/130 mm Hg.
Complications of the crisis:
- acute myocardial infarction;
- acute cerebrovascular accident;
- retinal hemorrhage;
- pulmonary edema
- cerebral edema;
- death.
In the long course of pheochromocytoma, signs of hyperactive metabolism are noted: weight loss, persistent fever. Also, due to an excess of catecholamines, patients often develop secondary diabetes mellitus.
Laboratory diagnostics of pheochromocytoma, instrumental studies
At the first stage of the diagnostic search, manifestations of excessive catecholamine production are detected. To do this, in addition to studying the symptoms, biological fluids (blood and urine) are examined for the presence of increased amounts of adrenaline and norepinephrine and metabolites. Their amount in pheochromocytoma exceeds the norm by three times or more.
To clarify the diagnosis, a conventional laboratory test is supplemented with an adrenolytic test with phentolamine, a drug that blocks the action of catecholamines. It is injected into the patient's vein: if after two minutes the blood pressure decreases by 25-35 mm Hg and this effect persists for 10-15 minutes, the reaction is considered positive.
After the first stage, the specialists proceed to the second stage - to find out the localization of pheochromocytoma on ultrasound, CT or MRI. The first method allows to detect an increase in the size of the adrenal glands, but is not able to detect a tumor. Computed tomography and MRI can detect even the smallest tumors and find out exactly where they are located. This is an indispensable condition for a future operation to remove pheochromocytoma.
In addition to these methods, selective angiography (tumors are permeated with a large number of vessels), radioisotope scanning and positron emission tomography are sometimes used. The latter methods are expensive and are used only in difficult diagnostic cases.
Signs of malignancy are difficult to establish with the available diagnostic methods. Even a histological examination of the removed specimen does not reveal malignancy of the tumor. The malignant course of the disease is determined by indirect data: aggressiveness of tumor growth in neighboring organs and tissues, the presence of metastases in the lymph nodes, bones, liver, and lungs.
Treatment of adrenal pheochromocytoma
The effectiveness of conservative treatment of this disease is very low. The cause of persistent hypertension is an excess of catecholamine production, and the use of medications does not help to get rid of it until this cause is eliminated. According to clinical guidelines, the only way to treat pheochromocytoma is surgery. Neither radiation nor chemotherapy can destroy the tumor.
The danger of surgery is that even a simple touch of the tumor can cause an explosive release of adrenaline and norepinephrine, which will cause a sharp rise and fall in blood pressure. Therefore, careful preoperative preparation is the key to a safe operation. The patient is prepared for several days (up to two weeks) by administering adrenergic-blocking drugs - phenoxybenzamine in a progressively increasing dose, prazosin, sodium nitroprusside, and less often - beta-blockers.
After achieving a stable pressure correction, the tumor is removed from all places where it has managed to grow. It is advisable to perform the operation in an open way, because during laparoscopy there is almost no opportunity to examine the abdominal cavity in detail.
Surgery to remove pheochromocytoma may not be performed in case of its massive growth into surrounding organs and tissues, metastasis of pheochromoblastoma (the so-called malignant form of the disease) or if the patient has severe concomitant diseases.
Prognosis.
With timely and high-quality treatment, hypertension in most cases disappears without a trace. Sometimes it remains, but it becomes less aggressive and better treatable with conventional drugs. In 10% of cases, relapses are possible due to the fact that the tumor can be located in places other than the adrenal glands. This requires additional treatment and possibly surgery.
Pheochromocytoma is a serious disease. Learn more on the website of our clinic Dobrodut.com. Our experts categorically do not recommend self-medication. Consult a good endocrinologist who will be able to prescribe the right treatment and resolve the issue of surgical removal of the tumor.
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