Kortikosteroma – a tumor of cortical substance of adrenal glands, independently sekretiruyushchy excess quantity of cortisol and leading to development of a syndrome of Itsenko-Cushing. Clinical manifestations of a kortikosteroma are characterized by simptomokompleks of a giperkortitsizm: arterial hypertension, kushingoidny obesity, muscular weakness, violation of carbohydrate exchange and sexual function, the virilizm phenomena (at women) and demaskulinization signs (at men). Diagnostics of a kortikosteroma includes polyposition ultrasonography, a stsintigrafiya, KT and MRT of adrenal glands, conducting deksametazonovy test, determination of level of cortisol of blood and urine. At detection of a kortikosteroma surgical treatment - an adrenalektomiya is required.
Kortikosteroma – the tumor of a puchkovy zone of cortical substance in excess quantities producing cortisol that is followed by clinic of an endogenous giperkortitsizm – Itsenko-Cushing's syndrome. Among a kortikoster in endocrinology benign (adenomas) and malignant (adenocarcinomas, kortikoblastoma) new growths meet. Than advanced age of the patient is less than the size of kortikosteroma, subjects a high probability of its good-quality character. Statistically, malignant kortikosteroma meet in 57% of cases, good-quality - in 43%; this type of a tumor is found in women by 5 times more often than at men.
Characteristic of a kortikosteroma
The majority a kortikoster develop in a puchkovy zone of bark of one of adrenal glands. Growth of a kortikosteroma from ektopirovanny nadpochechnikovy fabric in kidneys and ovaries is possible. The sizes of benign adenomas make 1-15 cm, weight - 2-200 . Good-quality kortikosteroma are covered with the dense capsule from connecting fabric with a plentiful vaskulyarization.
Adenocarcinomas and kortikoblastoma can reach the huge sizes and weight 800 - 1200 . Malignant kortikosteroma have a hilly dense surface with a large number of vessels; on a section of a tumor sites of necroses and hemorrhages because of what tumoral fabric gets motley coloring with alternation of zones of yellow and dark cherry color come to light. Infiltrative growth malignant a kortikster leads to germination of okolopochechny cellulose, the main vessels and defeat of the next bodies. Metastasis of a tumor occurs on lymphatic and blood ways to zabryushinny and paraaortal lymph nodes, lungs and a liver. Kortikosteroma metastasises also have hormonal activity and can continue to synthesize cortisol even after removal of the main tumor.
Reasons and pathogenesis of a kortikosteroma
The etiology and risk factors of tumors of adrenal glands are studied insufficiently. Among probable causes of development of a kortikosteroma heredity, aggressive factors of the environment, influence of separate types of medicines are considered. Hormonal activity of a kortikosteroma is shown in excess synthesis of glucocorticoids, to a lesser extent – mineralokortikoid and androgens. At surplus of glucocorticoids on a feedback mechanism synthesis of AKTG a hypophysis decreases owing to what intact tissues of adrenal glands are exposed to atrophic changes.
Biological effects of an endogenous giperkortitsizm at the fabric level are characterized by catabolic action on bone, muscular and connecting tissue that is followed by system dystrophic processes. Course carbohydrate (hyperglycemia), fatty and electrolytic exchange (a gipernatriyemiya and a gipokaliyemiya) is roughly broken owing to what there are steroid diabetes, obesity, arterial hypertension, heart failure, arrhythmias. Immunosupressivny action of glucocorticoids is shown by tendency to infections.
The clinical picture of a kortikosteroma is caused by the endogenous giperkortitsizm leading to changes from various bodies. To early and stable signs of a kortikosteroma kushingoidny obesity, arterial hypertension, a myasthenia, a headache, violation of tolerance belong to glucose. Violations of water and electrolytic exchange are shown by a polidipsiya, a polyuria, decrease in specific weight of urine.
On skin of mammary glands, a stomach, the internal surfaces of hips petekhialny hemorrhages, crimson and cyanotic striya develop. Pustulous rashes - eels, follikulita are frequent; on the lower extremities the centers of a hyperpegmentation and trophic ulcers are formed. Causes the virilization phenomena in women of a kortikosterom: girsutizm, decrease in a timbre of a voice, and , clitoris hypertrophy. Demaskulinization signs – a ginekomastiya, a hypoplasia of testicles, decrease in a potentiality are found in men with kortikosteromy.
At a considerable part of patients the osteoporosis which is quite often leading to pathological fractures of edges, compression spinal fractures develops. In a third of cases the current of a kortikosteroma is accompanied by chronic pyelonephritis and an urolithic disease. Development mentally of violations - excitement, a depression is possible. The malignant kortikosteroma, except Cushing's syndrome, is followed by intoxication characteristic of oncological tumors.
Diagnostics of a kortikosteroma
The leading role in diagnostics of a kortikosteroma belongs to definition of kortikosteroidny hormones and their metabolites in serum of blood and urine. For differentiation of a kortikosteroma with the AKTG-ektopichesky syndrome and Itsenko-Cushing's disease big deksametazonovy test (Liddl's test) with the subsequent definition of cortisol and AKTG is carried out. At a kortikosteroma after dexamethasone reception the level of cortisol of blood and an ekskretion 17-OKC do not decrease; increase in AKTG in plasma of blood is considered as manifestation of the AKTG-ektopichesky syndrome.
For the purpose of topichesky diagnostics of a kortikosteroma the complex of the visualizing researches is conducted: Ultrasonography of adrenal glands, abdominal cavity, kidneys, ovaries; KT and MRT of adrenal glands, a stsintigrafiya with 1311-19 cholesterol. For assessment of changes from other bodies and systems consultations of the cardiologist, the urologist-andrologist, the gynecologist, the neurologist will be organized.
Treatment and forecast of a kortikosteroma
Identification of a kortikosteroma serves as the absolute indication to surgical removal of a tumor. At a kortikosteroma removal of an adrenal gland (adrenalektomiya) is made by an open or endovideo surgical method. For prevention of nadpochechnikovy insufficiency in the early postoperative period replaceable hormonal therapy is carried out.
After removal of a malignant kortikosteroma for the purpose of suppression of hormonal activity of possible eliminations of a tumor inhibitors of secretion of corticosteroids are in addition appointed (). At clinically and radiological the confirmed metastasises carrying out chemotherapy is required.
Removal good-quality a kortikoster promotes gradual regress of clinical symptomatology: to change of appearance, normalization HELL and exchange processes, to a pobledneniye of striya, restoration of sexual function, disappearance of steroid diabetes, decrease in body weight, restoration of a bone tissue etc. In the postoperative period dispensary observation of the endocrinologist is shown to patients. At a malignant kortikosteroma the forecast unsuccessful.