A tumor of the adrenal cortex that produces an excess of cortisol and thus becomes the trigger of the Itzenko-Cushing syndrome is called a corticosteroma. The main symptomatology of the disease is Cushing’s obesity, which is accompanied by hypertension, muscle weakness, decreased sexual function, and carbohydrate metabolism disorder. In women, symptoms of virilism and demasculinization in men are additionally manifested. How the tumor is diagnosed and treated, you will learn from the article.
- Characteristics of the tumor.
- Causes and pathogenesis.
Corticosteroma is a neoplasm of the adrenal glands. Its main feature is the synthesis of cortisol in excess, which provokes endogenous hypercorticism or Icenko-Cushing’s syndrome. The tumor belongs to the family of hormone-dependent corticosteroid tumors, among which there are benign – adenomas and malignant – adenocarcinomas, corticoblastomas.
The older the patient and the smaller the neoplasm, the more evidence for its benignity. According to statistics, malignant tumors of the carcinoma family account for more than 50%, and the rest are benign masses. The tumor is gender-specific, occurring 5-fold more frequently in men, with no endemicity, seasonality, age, or racial features noted in the tumor.
The adrenal gland has several zones in its cortex, the corticosteroma grows from the bundle and affects one adrenal gland. In addition, the source of the tumor can be ectopic adrenal tissue in the kidneys and ovaries. The size of the benign variant reaches from 1 to 15 cm, weight – from 2 to 200 grams. The neoplasm itself is encapsulated by a connective tissue sheath, which is perfectly supplied with blood.
Malignant tumors are usually giants and weigh 800 to 1,200 grams. They have a dense, bumpy surface and many capillaries. On the section of the neoplasm you can see foci of necrosis and hemorrhage, so the tumor looks mottled: alternating loci of yellow and cherry shades. Malignant corticosteromas have infiltrative growth, which allows them to penetrate the peritoneal tissue, great vessels, and neighboring organs.
Metastasis occurs via lymphatic vessels, blood flow to retroperitoneal and para-aortic lymph nodes, lungs, and liver. Corticosteroma metastases are hormonally active and can produce cortisol even after excision of the main tumor.
Causes and pathogenesis
The triggers of adrenal tumors are not completely clear.
Among the main triggers are:
- aggressive external environment;
- insulin and leptin resistance leading to obesity.
Hormonal activity of the tumor is primarily an excess of glucocorticoid production, and slightly less of mineralocorticoids and androgens. Excess of the former by feedback type leads to minimization of the pituitary CCTH synthesis, which is expressed in atrophy of the adrenal glands. Leptin is involved in the pathological process indirectly, since the pituitary and adrenal glands are affiliated with the hypothalamus, where the centers of eating behavior are located.
Bioeffects of endohypercorticism at the tissue level are expressed by catabolism of bones, muscles, connective tissue, which results in systemic dystrophy. The quality of carbohydrate, lipid and electrolyte balance changes drastically. Hyperglycemia, hypokalemia and hypernatriemia arise, which is clinically manifested by steroid diabetes, obesity, hypertension, heart failure, heart rhythm disturbances. Immunosuppression of glucocorticoids causes susceptibility to infections.
The clinic of corticosteroma is varied, because endogenous hypercorticism changes the condition of almost all vital internal organs.
Signs of the tumor are considered to be:
- cushingoid obesity;
- high blood pressure;
- myasthenia gravis;
- impaired glucose and leptin tolerance;
- water-salt imbalance in the form of polydipsia, polyuria, reduced urine specific gravity;
- petechiae and skin striae, pyoderma, acne, folliculitis;
- trophic leg ulcers with hyperpigmentation.
Women are diagnosed with:
- changes in the timbre of the voice;
- menstrual irregularities;
- hypertrophy of the clitoris.
In men – demasculinization with gynecomastia, hypoplasia of testicles, decreased erection. Osteoporosis is often diagnosed, which causes pathological fractures and spinal compression. One third of patients are diagnosed with IBC, pyelonephritis, and there may be mental disorders. Corticosteroma is accompanied by intoxication, which allows to speak about its oncological nature.
The determination of corticosteroids and their metabolites in blood and urine is of primary importance for proper diagnosis. To differentiate between Icenko-Cushing’s syndrome and the disease of the same name, a large Liddle test is performed with dexamethasone followed by determination of cortisol and ACTH. In corticosteroid syndrome there is no drop in cortisol concentration in blood or urine after dexamethasone administration, but an increase in plasma ACTH is considered as a manifestation of ACTH-ectopic syndrome.
For the topical diagnosis of the tumor, a set of examinations is performed:
- Ultrasound, CT and MRI of the adrenal glands;
- Ultrasound of the abdominal cavity organs;
- Ultrasound of the kidneys;
- Ovarian ultrasound;
- Scintigraphy with 1311-19-cholesterol.
Changes in other internal organs and systems are detected by narrow specialists.
Corticosteroma is an absolute indication for its surgical removal. If the tumor is detected, adrenectomy is performed by open surgery or endovideosurgery. For prevention of organ insufficiency in the postoperative period hormone replacement therapy is prescribed. Corticosteroid secretion inhibitors like Mitotan are used for prevention of metastases, and chemotherapy is performed if foci of dropout are detected.
Removal of the benign variant of the tumor leads to regression of the symptoms of the pathology: the appearance of patients changes, blood pressure and metabolism stabilize, stretch marks become pale, potency restores, steroid diabetes disappears, extra pounds go away, bones become denser. After removal of the neoplasm patients are observed by an endocrinologist. With a malignant tumor, the prognosis is unfavorable.