Pheochromocytoma and hypertension
Synonyms in a broader sense
English: pheochromocytoma
definition
Pheochromocytoma is a disease in which a tumor produces too much hormones. This hormone-producing tumor is made up of cells derived from the stress-inducing nervous system. In 90% of the cases the tumor is in the Adrenal gland, in 10% it is in the range of Spine.
The pheochromocytoma forms the stress-relieving hormones adrenaline and Norepinephrine in large quantity and pour them into the Bloodstream out. Norepinephrine and adrenaline are so called Catecholamines.
Adrenaline causes blood vessels to constrict, while noradrenaline has a vasodilating effect and am Heart the increase in heart rate and increased punch. For the most part, the pheochromocytoma forms adrenaline, which is why vascular narrowing takes place and very often a high level in the affected patients Blood pressure occurs.
In the vast majority of patients, the pheochromocytoma is unilateral, in a few cases it is a bilateral hormone-forming tumor.
In 90% of cases the pheochromocytoma is a benign tumor, 10% of the tumors are malignant.
Pheochromocytoma can be the expression of a familial disease that is associated with tumors in various organs. This disease is called MEN 2 syndrome, where MEN stands for multiple endocrine neoplasias. It is a disease in which several (= multiple) endocrine (= hormonal) tumors (= neoplasias) occur.
Occurrence in the population
Every year, one person per 100,000 will develop a pheochromocytoma. In less than 1% of cases of high blood pressure diseases, the pheochromocytoma is responsible for the high blood pressure (arterial hypertension).
diagnosis
An increase in stress-relieving hormones in the body, as is the case with pheochromocytoma, can be diagnosed by a urine test:
The patient collects his urine in a specially prepared container for 24 hours. This urine is examined for the hormones themselves and the breakdown products of the catecholamines. If hormones or degradation products are found to a high degree, evidence is provided that there is an overproduction of hormones. Values above 200 ng (spoken: nanograms) per liter indicate a pathological change in the production of hormones in the body, while values below 50 ng represent a normal finding.
If you have a clinical suspicion of pheochromocytoma, drugs should be discontinued two weeks before the examination, which can falsify the diagnosis, such as Tetracyclines, theophylline or clonidine.
A test confirms the suspicion of a hormone-forming tumor:
A blood test determines the amount of catecholamines in the blood. The patient then receives the drug clonidine. The blood will be tested again for catecholamines. In healthy people, the clonidine causes a decrease in the amount of catecholamine in the blood, in patients with pheochromocytoma, however, the amount remains unchanged. This effect occurs because clonidine inhibits the normal production of catecholamines in the adrenal gland. The pheochromocytoma is decoupled from the body's hormonal control system; the tumor forms too much hormones independently and independently of the body's normal needs. As a result, the hormone production cannot be inhibited by the drug administered.
Various examination methods such as ultrasound, computed tomography (CT) or imaging using magnetic resonance therapy (MRT) are used to localize the location of the tumor in the body.
The patient should also be examined for the presence of a MEN-2 syndrome: New growths of the thyroid gland and the parathyroid gland, such as those present in MEN-2 syndrome, must be clarified with the help of examinations.
You might also be interested in this topic: Conn syndrome
Symptoms
Typical symptoms at Pheochromocytoma are a high blood pressure (see also: Increase in the second blood pressure value), a headache, noticeable Palpitations (Palpitations), sweating, nausea and vomiting, and panic attacks.
The discomfort can be caused by exertion, emotional stress or pressing on the stomach.
The patients often notice an inner restlessness. Your skin is quite pale and when you see a doctor you say you have lost weight recently. In a third of the cases there is an increased blood sugar level (Diabetes mellitus) and the increased excretion of sugar in the urine.
therapy
Surgical removal of the tumor is the treatment of choice and regular follow-up examinations over the next five years. Before the operation and for a certain period after the operation, the patient must be treated with medication to prevent a sharp drop in blood pressure after the tumor has been removed.
If an operation is not possible, drug therapy is also conceivable: The patients either receive an alpha blocker, which inhibits the effect of the catecholamines, or alpha-methyl-p-tyrosine, a drug that prevents the formation of catecholamines.
When the excessively high amount of hormones drops to normal values, the blood pressure is regulated down again and 80% of patients with high blood pressure achieve normal blood pressure values. The other 20% of patients have primary high blood pressure that was not caused by the increased hormone production and therefore continues to exist.
forecast
The benign pheochromocytomas can recur in 5-10% of cases, which is why follow-up examinations should be carried out at regular intervals in order to detect a new tumor at an early stage.
For malignant tumors, follow-up examinations are also very important and should definitely be carried out.
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