What is pheochromocytoma, the symptoms, diagnosis and tactics of tumor treatment

1 Medical indications

Pheochromocytoma is more often detected in women aged 30-50 years. In 10% of cases, pathology is found in children. Malignant pheochromocytomas are pheochromoblastomas of the adenocarpal localization. If the disease is hereditary, then it is combined with the disease of Recklinghausen.

The main pathology negatively affects the vessels, heart, nerve cells and endocrine system. Adrenaline stimulates alpha-2 and beta-2-adrenergic receptors. The influence of the hormone is associated with an increase in heart rate and other indicators of the main body.

The first symptoms of the disease are manifested in the form of tachycardia, dilated pupil, profuse sweating, polyuria. Pheochromocytoma is a hormonal-active adrenal tumor that disrupts hemodynamics. Given the clinical course, specialists distinguish between paroxysmal, persistent and mixed forms of the disease.

In the first case, the patient is worried about a sharp increase in blood pressure, migraine, dizziness, a constant sense of fear, pale skin, chest and abdominal pain. The catecholamine crisis provokes microcytosis, lymphocytosis, and glucosuria. The attack manifests itself against the background of physical stress, overeating or abuse of alcohol, stress. The duration of this state is from a few minutes to 2-3 hours.

Symptoms of persistent pheochromocytoma manifest themselves as a persistent increase in blood pressure. In mixed form, the patient complains of frequent pheochromophytomic crises. A severe course of the disease is characterized by a disorderly change of low and high blood pressure. This condition threatens the patient's life.

2 Patient examination

Laboratory diagnosis helps to evaluate the production and secretion of catecholamines. The accuracy of the technique reaches 96%.At the same time, the patient is recommended to donate blood to determine the level of adrenaline, dopamine and norepinephrine. The advantages of determining catecholamines in the blood include the possibility of frequent study.

Determination of catecholamines in urine allows assessing the secretory activity of a tumor over a certain period. To increase the effectiveness of diagnostic procedures, pharmacological tests are used. They are based on the ability of certain drugs to block or provide release of the tumor by catecholamines.

The effective diagnostic procedures for pheochromocytomas are MRI and CT.Sensitivity of ultrasound in relation to pheochromocytomas varies within 90%.This indicator increases if the size of the lesion exceeds 2 cm. To assess the condition of the adrenal glands, selective phlebography is shown.

This disease is often differentiated with hypertension. Since the clinic of these pathologies has some similarities, the doctor draws attention to the following for the accurate diagnosis:

• the age of the patient - hypertension develops more often after 40 years;
• concomitant diseases - pheochromocytoma provokes the development of hypertension in children.

Hypertension, which occurs with diabetes mellitus, is suspicious of the presence of the pathology in question. This suspicion increases if insulin does not affect hyperglycemia.

3 Methods of therapy

Radical treatment of the ailment in question consists in surgical removal of the tumor. Preliminarily the patient is prepared for the forthcoming operation. The preparation is aimed at stabilizing the hemodynamic parameters. To normalize blood pressure, a-adrenoblockers are used( Tropafen, Fentolamin).Successful treatment is indicated by the appearance of an orthostatic reaction in the patient when the position of the body changes.

With a mixed form, it is possible to normalize blood pressure with the help of Catapressan, Raunatin, and Apressin. If the patient has a paroxysmal pheochromocytoma, hypotensive drugs are contraindicated. Adrenoblockers are appointed taking into account the possibility of collapse. More often patients are prescribed Prazosin. With the help of an elective blockade of postsynaptic receptors, tachycardia can be prevented.

When preparing for surgery, the doctor monitors the general condition of the patient. To assess the main hemodynamic indicators, the integral rheography of the body is used. The choice of the method of carrying out the operation depends on ensuring maximum freedom of manipulation in the wound. To remove the tumor, doctors use the following methods:

• extraperitoneal;
• transperitoneal;
• combined;
• Transthoracic.

Some patients have no normal hemodynamic parameters after surgery.

High or low blood pressure can be associated with a renal artery bandaged during surgery, with partial removal of malignant neoplasm, concomitant hypertension of the essential type.

The presence of hypotension indicates a renewed bleeding, an increase in the capacity of the venous bed.

If a multiple pheochromocytoma is identified, then several surgical interventions are indicated. Refusal of such prolonged treatment is allowed if:

• tumor is not removed;
• surgery represents a risk to the life of the patient.

The operation is divided into several stages if a multicentric pheochromocytoma growth is detected or a radical operation is contraindicated.

4 Preparatory stage

To reduce hyperkatecholamineemia, doctors use a-methyl tyrosine. The effect of this drug is aimed at reducing the concentration of catecholamines. In 80% of cases, a positive effect is observed. In other patients, the drug provokes mental and extrapyramidal disorders, diarrhea, various abnormalities in the work of the digestive tract.

These patients are assigned a-methylparathyrosine. This medicine reduces the release of catecholamines into the blood. With this drug, you can reduce the risks for the patient to perform surgery. If the disease lasts for a long time, then the following medicines are taken before the operation:

• alpha-blockers for prolonged exposure - prescribe Phenoxybenzamine, the dosage of which gradually increases, then selective Prazosin;
• beta-blockers - prescribe against a sufficient blockade of alpha-adrenergic receptors to prevent and eliminate supraventricular arrhythmia, prescribe propranolol or metoprolol.

If the patient is in serious condition or has metastasis of malignant neoplasms, Metirozine( tyrosine hydroxylase inhibitor) is used. Less often hemodynamics stabilizes with labetalol, captopril, and diphdepine. With malignant pheochromocytoma, combined treatment with Vincristine, Cyclophosphamide and Dakarbazine is indicated. If the doctor diagnosed "metastasizing pheochromocytoma and secreting VIP with calcitonin," then take Somatostatin.

5 Disease prognosis

Radical therapy contributes to the almost complete recovery of most patients. Only 5-12% of cases are diagnosed with relapse. But prolonged hyperkatecholamineemia, a secondary change in the functioning of the cardiac or vascular system and the kidneys, contributes to the preservation of some signs of pheochromocytoma. The recurrence risk group includes children and patients with ectopic or multiple tumors.

The prognosis of patient survival depends on the presence or absence of metastases. The remaining signs of the tumor, including angioinvasia and the germination of the capsule, are less reliable in this case. According to statistics, cure after surgery is diagnosed in 90% of cases.

To patients with pheochromocytoma, surgical treatment is vital. But after it, most patients have AH.Such patients are under dynamic dispensary supervision. The prognosis is generally positive, including cases with pheochromoblastoma( if there are no distant metastases).Tachycardia develops in 50% of patients.