Corticosteroma is a tumor of the adrenal cortex that autonomously secretes an excessive amount of cortisol and leads to the development of Itsenko-Cushing syndrome. Clinical manifestations are characterized by a symptom complex of hypercorticism: arterial hypertension, kushingoid obesity, muscle weakness, impaired carbohydrate metabolism and sexual function, virilism phenomena (in women) and signs of demasculinization (in men). Diagnosis includes polypositional ultrasound, scintigraphy, CT and MRI of the adrenal glands, dexamethasone testing, determination of blood and urine cortisol levels. When corticosteroma is detected, surgical treatment is required – adrenalectomy.
Meaning
Corticosteroma is a tumor of the bundle zone of the cortical substance, producing cortisol in excessive amounts, which is accompanied by a clinic of endogenous hypercorticism – Cushing syndrome. Among corticosteroids in endocrinology there are benign (adenomas) and malignant (adenocarcinomas, corticoblastomas) neoplasms. The older the patient’s age and the smaller the size of the corticosteroma, the greater the likelihood of its benign nature. According to statistics, malignant corticosteromas occur in 57% of cases, benign – in 43%; in women, this type of tumor is detected 5 times more often than in men.
Characteristic
Most corticosteroids develop in the bundle zone of the cortex of one of the adrenal glands. Corticosteroma may grow from ectopic adrenal tissue in the kidneys and ovaries. The dimensions of benign adenomas are 1-15 cm, weight – 2-200 gr. Benign corticosteromas are covered with a dense capsule of connective tissue with abundant vascularization.
Adenocarcinomas and corticoblastomas can reach gigantic sizes and a weight of 800 – 1200 grams. Malignant corticosteromas have a lumpy, dense surface with a large number of vessels; areas of necrosis and hemorrhage are detected on the tumor section, which is why the tumor tissue acquires a mottled color with alternating zones of yellow and dark cherry color. The infiltrative growth of malignant corticosteroids leads to the germination of the perinephrine fiber, the main vessels and damage to neighboring organs. Tumor metastasis occurs along the lymphatic and circulatory pathways to retroperitoneal and paraaortic lymph nodes, lungs and liver. Corticosteroma metastases also have hormonal activity and can continue to synthesize cortisol even after the removal of the main tumor.
Causes and pathogenesis
The etiology and risk factors of adrenal tumors have not been sufficiently studied. Among the probable causes of the development of corticosteroma, heredity, aggressive environmental factors, and the effects of certain types of drugs are considered. The hormonal activity of corticosteroma is manifested in the excessive synthesis of glucocorticoids, to a lesser extent – mineralocorticoids and androgens. With an excess of glucocorticoids, the synthesis of ACTH by the pituitary gland decreases by the feedback mechanism, as a result of which intact adrenal tissues undergo atrophic changes.
The biological effects of endogenous hypercorticism at the tissue level are characterized by a catabolic effect on bone, muscle and connective tissue, which is accompanied by systemic dystrophic processes. The course of carbohydrate (hyperglycemia), fat and electrolyte metabolism (hypernatremia and hypokalemia) is grossly disrupted, resulting in steroid diabetes, obesity, hypertension, heart failure, arrhythmias. The immunosuppressive effect of glucocorticoids is manifested by a tendency to infections.
Symptoms
The clinical picture of corticosteroma is caused by endogenous hypercorticism, leading to changes on the part of various organs. Early and stable signs of corticosteroma include kushingoid obesity, hypertension, myasthenia gravis, headache, impaired glucose tolerance. Violations of water-electrolyte metabolism are manifested by polydipsia, polyuria, and a decrease in the specific gravity of urine.
Petechial hemorrhages, purplish-cyanotic striae appear on the skin of the mammary glands, abdomen, and inner surfaces of the thighs. Pustular rashes are not uncommon – acne, folliculitis; foci of hyperpigmentation and trophic ulcers form on the lower extremities. In women, corticosteroma causes virilization phenomena: hirsutism, a decrease in the timbre of the voice, dysmenorrhea and amenorrhea, hypertrophy of the clitoris. Men with corticosteroma show signs of demasculinization – gynecomastia, testicular hypoplasia, decreased potency.
A significant part of patients develop osteoporosis, which often leads to pathological fractures of the ribs, compression fractures of the spine. In a third of cases, the course of corticosteroma is accompanied by chronic pyelonephritis and urolithiasis. It is possible to develop mental disorders – arousal, depression. Malignant corticosteroma, in addition to Cushing’s syndrome, is accompanied by intoxication, characteristic of oncological tumors.
Diagnostics
The leading role in the diagnosis of corticosteroma belongs to the determination of corticosteroid hormones and their metabolites in blood serum and urine. To differentiate corticosteroma with ACTH-ectopic syndrome and Itsenko-Cushing’s disease, a large dexamethasone test (Liddle test) is performed, followed by the determination of cortisol and ACTH. With corticosteroids, after taking dexamethasone, the level of cortisol in the blood and the excretion of 17-ACS do not decrease; an increase in ACTH in blood plasma is considered as a manifestation of ACTH-ectopic syndrome.
For the purpose of topical diagnosis of corticosteroma, a complex of imaging studies is carried out:
- ultrasound of the adrenal glands, abdominal cavity, kidneys, ovaries;
- CT and MRI of the adrenal glands,
- scintigraphy with 1311-19-cholesterol.
Consultations of a cardiologist, urologist-andrologist, gynecologist, neurologist are organized to assess changes on the part of other organs and systems.
Treatment
Detection of corticosteroma is an absolute indication for surgical removal of the tumor. With corticosteroma, the adrenal gland is removed (adrenalectomy) by an open or endovideosurgical method. Hormone replacement therapy is performed to prevent adrenal insufficiency in the early postoperative period.
After removal of a malignant corticosteroma, in order to suppress the hormonal activity of possible tumor dropouts, corticosteroid secretion inhibitors (mitotan) are additionally prescribed. With clinically and radiologically confirmed metastases, chemotherapy is required.
Forecast
Removal of benign corticosteroids contributes to a gradual regression of clinical symptoms: a change in appearance, normalization of blood pressure and metabolic processes, paling of striae, restoration of sexual function, disappearance of steroid diabetes, weight loss, restoration of bone tissue, etc. In the postoperative period, patients are shown a dispensary observation of an endocrinologist. With malignant corticosteroma, the prognosis is unfavorable.