Pheochromocytoma - a hormonally active adrenal gland tumor. It is located in the medulla or hromaffinovoy gland tissue and is considered one of the most poorly known endocrine abnormalities.
Many aspects of the etiology and development of adrenal pheochromocytoma modern medicine are still unknown. The familial form of pheochromocytoma is typical for people with hereditary diseases: Reklingauzena disease (also known as neurofibromatosis) syndrome or Sipple Hippel-Lindau.
Pheochromocytoma occurs, on average, 1 out of 10 thousand. Man. Among those suffering from hypertension, the incidence is 1%, ie, 1 person is already on 100 patients with high blood pressure.
The difficulty in diagnosing adrenal pheochromocytoma is often asymptomatic disease or in a wide range of clinical symptoms of pheochromocytoma. So according to the American physicians correctly diagnosed patients with pheochromocytoma is established only in 37-40% of cases.
Typically, adrenal pheochromocytoma located in one of the glands. The defeat of the two pairs of tumor localization of pheochromocytoma or adrenal region is diagnosed only at 10-15% of diseases.
And yet 10% of malignant pathology detected symptoms of pheochromocytoma - metastasis to lymph nodes, bone, muscle, liver, at least - in the lungs and brain.
Symptoms of adrenal pheochromocytoma
One of the main symptoms of pheochromocytoma is hypertension. High blood pressure in a patient can be episodic or chronic. In the first case of hypertension attacks provoke emotional distress, excessive exercise or overeating.
The symptoms of pheochromocytoma during an attack of hypertension include:
- headache pulsating nature,
- pale skin,
- discomfort in the chest and abdomen,
- cramping calf muscles.
After the attack, the patient has the complete disappearance of all symptoms of pheochromocytoma, a sharp drop in blood pressure up to the radically opposite state - hypotension.
Differential diagnosis of pheochromocytoma should be performed in people with complaints:
- anxiety attacks,
- hyperventilation syndrome,
- hot flashes during menopause,
- increased need for caffeine,
- brief loss of consciousness.
Among the symptoms of pheochromocytoma complicated forms isolated signs of neuropsychiatric, cardiovascular, endokrinno- metabolic, hematological and gastrointestinal disorders:
- heart failure,
- kidney and vascular lesions of the fundus,
- hyperglycemia (high blood glucose)
- hypogonadism (Hormone-androgen)
- increase in erythrocyte sedimentation rate or blood
- drooling, etc.
Diagnosis of adrenal pheochromocytoma
Clinical symptoms of pheochromocytoma is the presence in the tissues of the urinary system, the adrenal catecholamines and bronchi. These specific substances produce kateholaminprodutsiruyuschie tumor tissue. Granules of catecholamines in the diagnosis of pheochromocytoma can be detected using the method of silvering Gamperlyu-Masson and Grimeliusu.
At the heart of the laboratory diagnosis of pheochromocytoma is an assessment of the secretion of catecholamines in a daily urine sample. In addition, the urine of the patient is often found adrenaline and vanillylmandelic acid.
The second stage of diagnosis of pheochromocytoma adrenal glands - the study of characteristics of the patient's blood pressure fluctuations. Patients with this type of hormone-dependent tumors usually found increased total vascular resistance and decrease of the volume of blood.
Compulsory stage diagnosis of pheochromocytoma is to control cardiac activity. A characteristic symptom of pheochromocytoma is the development of cardiomyopathy and sustained heart rhythm disorder.
After the clinical and laboratory tests carried out topical diagnosis of pheochromocytoma - scintigraphy with radioactive metayodbenzilguanidinom. Effective method of diagnosis of pheochromocytoma with 90-100% susceptibility to tumors are also MRI and CT.
Use of ultrasonography in the diagnosis of pheochromocytoma is justified only if there is a tumor more than 2cm. Angiography in the diagnosis of pheochromocytoma is rarely used due to the time consuming method and the high risk of complications.
Treatment of adrenal pheochromocytoma
The most radical method of treatment of pheochromocytoma - surgical removal of the tumor. However, it can only be carried out after stabilization of arterial pressure.
To this end, preoperative treatment of pheochromocytoma patient is assigned a reception-blockers: phenoxybenzamine, phentolamine, Tropafen etc. The criterion for the effectiveness of treatment of pheochromocytoma is the appearance of orthostatic blood pressure fluctuations caused by the change in body position.
The choice of method of surgical treatment of pheochromocytoma depends on the characteristics of the tumor. Good manipulation during surgery provide transbryushinny, transthoracic, extraperitoneal or combined approaches to surgery.
When single tumor efficacy of surgical treatment of pheochromocytoma is high. Relapse observed only in 12-15% of cases.
In some cases, after removal of the tumor in the patient is no reduction in pressure. Such postoperative complication associated with damage to the renal artery or incomplete removal of the tumor.
When multiple tumors unified standard in the surgical treatment of pheochromocytoma exists. Appropriately considered complete removal of all tumors, however, due to high-risk operations from the tactic of treatment of pheochromocytoma have to give, and resection of tumors in several stages, or remove only a portion of the tumor.
Conservative treatment of adrenal pheochromocytoma less effective. It aims to reduce the body's levels of catecholamine drugs based on A-metiltirozinoma. Conservative treatment of pheochromocytoma is able to reduce by 80% the amount of catecholamines and prevent the development of hypertensive crisis. However, a systematic technique A metiltirozinoma can lead to mental disorders and functional disorders of the gastrointestinal tract.