Endocrinology / / August 12, 2017
tumors that produce catecholamines, can be derived from the adrenal medulla and from ekstraadrenalovoy hromaffinovoy tissue.To identify tumors of paraganglia existed, the term "paraganglioma", but now decided to combine the hormonal activity of the tumor tissue hromaffinovoy called pheochromocytomas with an indication of their origin - adrenal or extraadrenal.
question about the etiology of tumor tissue hromaffinovoy is part of the overall problem of the origin of tumors.
There is evidence of a genetic predisposition to develop pheochromocytomas.In this case the tumor to develop in people who have a tendency to the earlier development of pheochromocytoma are sick, more often bilateral or multiple, and the disease among relatives.
Experimental studies indicate a possible role in the origin of pheochromocytomas of hormonal balance.Pheochromocytomas were obtained in rats and guinea pigs when administered to them estrogens and growth hormone.
Pathogenesis The pathogenesis of painful man
presence of crises inherent in pheochromocytoma, and in particular the emergence of crises after trauma, making it likely the value of the central nervous system in their origin.
Phaeochromocytomas are benign or malignant.
malignant pheochromocytoma according to most statistics account for about 10% of these tumors.However, taking as a criterion for the presence of malignancy is hormonally active metastases, malignant tumors of the adrenal medulla are less common, accounting for 2.8% pheochromocytomas in adults and 2.4% - in children.
When bilaterally or ekstraadrenalovom location pheochromocytomas are more often malignant than in the case of tumor growth from one of the adrenal gland.
Phaeochromocytomas usually develop from the adrenal medulla, hitting one adrenal gland, but about 10% tumor - bilateral or multiple.In 5-10% of cases the tumor develops from paraganglia or sympathetic ganglia and is located along the major vessels of the abdominal cavity, in the sympathetic ganglia.Rarely described hilar localization of pheochromocytoma, its location in the soft tissues of the neck, the bladder.Ekstraadrenalovoe location of the tumor and multiple lesions more common in children and in people with a family history of the disease.
Dimensions pheochromocytomas usually 1-15 cm, weight - from 1 to 75 g Sometimes there are large tumors up to 3600, the benign pheochromocytoma encapsulated are solid or cystic structure.The consistency of the tumor is usually mild.Characterized by a large number of foci of hemorrhage and necrosis.Cystic tumors have hemorrhagic content.Richly vascularized tumor.
Pheochromocytoma is constructed mainly of large polygonal cells.Some of them at fixing chromium salts colored in yellow-brown color.The nuclei are polymorphic cells.In the cytoplasm of the cell and outside there are hyaline inclusions.Among conventional tumor cells are observed with large ovoid cell cytoplasm and clear well dyeable core without giving reaction with chromium salts.
malignant pheochromocytoma cell atypia characterized by large, germination capsules and blood vessels, metastasis to the lymph nodes, liver, bones and lungs.
Pheochromocytoma - a rare tumor.At half-sectional statisticians incidence of the disease is 3.6 cases per 10 000 dead.Among patients with hypertension, pheochromocytoma frequency is 2-3 times higher.The disease is more common in middle age, but it also happens in children and the elderly.Children are more common genetically determined form and, unlike adults who have not revealed sexual differences in the frequency of the disease, are more common in boys.
Clinical manifestations of pheochromocytoma are highly variable.The most typical manifestation of the disease is high blood pressure.Hypertension may be permanent or paroxysmal.In some patients, there are crises with increased blood pressure in the background of a permanent hypertension.
crisis, characteristic of pheochromocytoma are characterized, in addition to a significant increase in systolic and diastolic blood pressure, a variety of neuro-vegetative disorders - headaches, sweating, palpitations, increased heart rate, rarely - bradycardia, pallor or redness of the face and lips, nervousness, excitement and a sense of fear, tremor, nausea and vomiting, pain in the chest, abdomen, lumbar region, paresthesias, and pain in the hands, visual impairment, hyperthermia.During the attack sometimes develops a coma.Often, there is shortness of breath, swelling of the neck veins and other symptoms of heart failure.Pupils are often extended.In some patients in connection with the violation of the metabolic processes in the myocardium during the crisis there is a violation of the rhythm of cardiac activity - extrasystole, paroxysmal tachycardia, conduction abnormalities.At the moment of attack often increases the number of white blood cells and red blood cells, there is a transient eosinophilia, and lymphocytosis.The blood sugar level rises, there is glycosuria.Increases in the number of blood neesterofitsirovannyh fatty acids.In the urine - a transient albuminuria and cylindruria.
All of these symptoms, except for hypertension, which is always the case at the time of crisis, is not binding and can be combined in various combinations.
In the initial period of the disease in some patients, crises are rare - once every several months or even years, uchaschayas as the disease progresses.Crises can be triggered by emotional arousal and physical overexertion, abundant meal, compression of the tumor during its palpation.Kriza duration from a few minutes to several hours, rarely - more.After the crisis is often excessive urination, severe weakness noted.
Crease can be complicated by bleeding in the retina, cerebrovascular, pulmonary edema and lead to death.
Along with paroxysmal or persistent hypertension in patients with pheochromocytoma have a tendency to a sharp drop in blood pressure up to collapse under stress.Frequently a marked decrease in blood pressure during the transition from horizontal to vertical.
addition to cardiovascular and neuro-vegetative manifestations, in connection with the metabolic action of catecholamines in patients with pheochromocytoma may develop a number of metabolic disorders.Hyperglycemia and glycosuria may be observed is crises, leading to the development of resistant diabetes.In many patients, body weight changes - often marked weight loss, at least - obesity.
In some cases, there are symptoms of hyperfunction of the adrenal cortex - hypertrichosis, and, sometimes, a picture, a close Syndrome Cushing - Cushing.These disorders can be explained by the stimulating effect of adrenaline on the production of ACTH by the anterior pituitary.
Some patients with pheochromocytoma occurs without obvious clinical manifestations;however, it can cause sudden death under stress due to the instability of the vascular or bleeding in the tumor.In a retrospective analysis of these patients is sometimes possible to establish the existence in the past mikrosimptomatiki - weakness, headaches, pain in the lumbar region.
- Physiology of the adrenal cortex
- Cushing syndrome - Cushing
- diagnosis and course of the syndrome Cushing's
- Treatment syndrome Cushing's
- androgen-producing tumors of the adrenal cortex
- Primary aldosteronism (Conn's syndrome)
- Biological effects of catecholamines
- feminizing tumor of the adrenal cortex
- Diseases of the adrenal medulla
- Treatment of chronic adrenal insufficiency
- congenital (inborn) hyperaldosteronism
- congenital virilizing adrenal hyperplasia
- Treatment of congenital adrenal hyperplasia
- Acuteadrenocortical insufficiency
hormonally active malignant pheochromocytoma (feohromoblastomy) along with the symptoms associated with increased production of tumor catecholamines, can manifest symptoms, generally characteristic of malignant tumors - cachexia, fever, accelerated erythrocyte sedimentation rate, and various pathological changes in theorgans where the metastasis has occurred.
clinical features of pheochromocytomas in children is the predominance of forms of the disease, the current from the constant rise in blood pressure and early development of complications associated with hypertension, in particular the destruction of the retina.
pheochromocytoma Complications include cardiovascular disorders associated with the presence of high blood pressure - increase in heart size, the development of circulatory failure, nephrosclerosis with renal impairment, retinopathy with hemorrhages and exudation.In patients with pheochromocytoma is often observed early development of atherosclerosis with coronary vessels.