Androsteroma

Androsteromas - virilizing tumors - belong to a rare pathology (1-3% of all tumors); virilizing, hormone-active tumor of the adrenal cortex, proceeding mainly from its reticular zone, excessively secretes corticosteroids, but mainly androgens, and characterized by a clinical picture similar to congenital adrenal cortex dysfunction. Androsteromas can reach large sizes - up to 1000-1200 g.

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Epidemiology

Androsteroma disease can occur at any age, both in women and men. Mostly women are ill, mostly up to 35 years old. The indication of researchers for rare androsteromas in men may be due to the difficulty in diagnosing - in adult men, virilization is less noticeable and, apparently, the part of the androster passes under the guise of hormone-inactive adrenal tumors.

Pathogenesis

The pathogenesis of androsteroma is due to the increased production of hormones, mainly androgens, tumor tissue. In pathoanatomical research, a tumor of the adrenal cortex is detected. Usually it is of a soft consistency, encapsulated. Histologically, tumors are mainly found in dark cells characterized by pronounced polymorphism. In a number of cases, the histological picture of the tumor resembles the structure of the mesh cortex of the adrenal cortex. With malignant androsterom, marked polymorphism, cellular atypia, infiltrative growth of tumor cells, and a variety of foci of necrosis are noted. In malignant androsteromas, metastasis can occur in the retroperitoneal space, the liver, and the lungs.

Androsteroma is usually widely encapsulated, the surface of the cut is red-brown, often with foci of necrosis, hemorrhage and cystic-altered areas. In tumors, one or more enzyme defects are usually detected, which cause the formation of excess amounts of androgens by the tumor tissue. Cells are compact, of ordinary size with vesicular nuclei; they form strands and acinar structures. In malignant tumors marked nuclear and cellular polymorphism and atypism are noted. Such cells form syncytial and alveolar structures, there is a discomplexed type of structure. Mitoses are rare. In children, virilizing tumors, usually malignant.

Symptoms of the androsteromas

The clinical picture in patients with androsteroma is caused by excessive production of the male sex hormones - androgens. The degree of virilization depends on the hormonal activity of the tumor, the duration of the disease. In female patients, androsteroma disease is manifested by virilization of varying degrees. The subcutaneous fat layer decreases, the musculature is released, its mass increases, the voice coarsens, becomes low; there is a growth of hair on the body and limbs; on the face - in the form of a beard and mustache, while on the head they fall out, a bald spot is formed. The mammary glands undergo a more or less pronounced atrophy. Menstruations begin to get confused and soon stop. Characteristic changes in the external genitalia - a significant increase and virilization of the clitoris. Patient well-being in the early stages of the disease remains good. Physical strength and performance may even increase (the action of androgens).

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Diagnostics of the androsteromas

With the purpose of visualization of the adrenal glands, RKT is used, with more often one-sided adrenal tumor. Data of RTD are of decisive importance in the diagnosis of androsteremia. Differential diagnosis should be carried out with bilateral virilizing hyperplasia of the adrenal glands. For this, samples are taken with the introduction of ACTH and dexamethasone. In connection with a certain autonomy of the tumor, i.e., independence of its function from the regulatory influence of the pituitary gland, the administration of ACTH and dexamethasone is not accompanied by a change in the excretion of 17-CS. X-ray examination (suprarenography) is combined with retrograde or infusion pyelography. Of the other diseases accompanied by virilization, one should remember about ovarian tumors producing androgens, adrenoblastomas. In these cases, excretion of 17-CS is not altered or moderately increased inadequately to the degree of virilization. The method of diagnosis is gynecological examination and X-ray diagnostics (oxypelviography).

The increase in the ovary on one side gives reason to suspect a tumor. Individual cases of the development of the viril syndrome on the soil of the lipoidicular ovarian tumor in an ectopic adrenocortical tumor in the ovary are described. The same diagnostic tests are used, including genicography. When hormonal studies in women with ovarian tumors, an increase in the secretion of androsterone, etiocholanolone, pregnan triol and testosterone-glucuronide is determined. With the introduction of metapirone or chorionic gonadotropin, there is a marked increase in these steroids. In boys, androsteroma should be distinguished from premature puberty associated with central nervous system damage, epiphysis  tumor, testicular tumor. With all this pathology, the excretion of 17-ketosteroids does not reach such a high level as with androsterom. At intracerebral processes there is a neurologic symptomatology.

Despite the seemingly vivid clinical picture of the disease, the diagnosis of androsteroma is often put late, and patients for several years are treated for ovarian dysfunction, alopecia, constitutional hirsutism, etc. In the case of children, there comes, in addition to the described symptoms, premature physical and sexual development in girls heterosexual, and boys in the isosexual type. Bone age is faster than the passport age. As a result of early closure of the growth zones, the patients remain undersized after treatment. Body weight usually does not increase, sometimes weight loss is noted. In the later stages, there is exhaustion, weakness, pain can arise due to the large size of the tumor.

Patients with androsteroma do not show those large metabolic disorders, as in patients with glucosteroma. The number of androgens, determined in urine and in blood, sometimes increases tens of times.

The Androsteroma Case

Patient GN, born in 1987, appealed to the endocrinologist of the RKB RKB in the summer of 2011 with complaints of hair growth throughout the body during the last 2-3 years, absence of menstruation during the last three months, weight loss by 10 kg for the last 2 years, increased sensitivity, inexplicable aggression, lability of mood, constant weakness.

She considers herself ill about 2-3 years, when she began to notice a decrease in weight against a background of good appetite, the appearance of increased growth of hair on the body, irregular menstrual cycle. The last few months, these symptoms worsened, menstruation completely stopped. To the doctor has addressed for the first time.

Heredity in endocrinological diseases is not burdened. Gynecological anamnesis: menstruation from the age of 14, regular, ungrowing, painless. Currently, amenorrhea. There were no pregnancies.

On examination, the condition is satisfactory. Height - 168 cm, weight - 52 kg, BMI - 18 kg / m2. The constitution is normostenic. Hair growth by male type. The skin is dry. Visible mucous membranes are clean, pale, there is no hyperpigmentation. The thyroid gland is not palpable. In the lungs, the breath is vesicular, there is no wheezing. The boundaries of relative dullness of the heart within the age limit. Heart rate is increased (96 beats / min.), Heart sounds are muffled. Blood pressure 100/60 mm Hg. Art. (in the sitting position). Orthostatic test is negative. Pulse of satisfactory filling, there is no pulse deficit. The abdomen is soft, painless. The liver is not enlarged, the dimensions according to Kurlov are 9x8x7 cm. The spleen is not enlarged. Peripheral edema, pasty there.

Results of the study

DGEA-C = 1460.7 (N-80.2-511.7 μg / dl), Testosterone = 13.4 (N 0.17 4.13 nmol / L), Cortisol = 8.2 (N 3.7 - 24.0 μg / dl), ACTH = 14.2 (N-0-46 pg / ml). The content of LH, FSH, STH, 17-hydroxyprogesterone, estradiol, somatomedine-c within normal limits.

Computer tomography is a multiple knotty formation of both adrenal glands. Adenomas. At a histological examination of the biopsy specimen, a dark cell adenoma of the adrenal cortex with massive hemorrhage. Ultrasound of the uterus and appendages - the condition corresponds to hypoestrogenia.

Based on anamnesis and clinico-laboratory data, a clinical diagnosis was made: Androsteroma of both adrenal glands.

The patient underwent adrenalectomy on the left and resection of the right adrenal gland. During the month of the postoperative period, the patient notes the restoration of the menstrual cycle, weight stabilization, mood stabilization, and qualitative improvement in well-being. Results of the postoperative examination: DHEA-C = 161.3 μg / dl, Testosterone = 1.27 nmol / l.

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Differential diagnosis

Differential diagnosis should be performed with congenital adrenal cortex dysfunction, Stein-Leventhal syndrome, virilizing  ovarian tumor  in women, in men and boys with testicular cancer, and with other types of premature puberty. In view of the fact that half, according to our data, and according to some observations, and more, malignant androster, important issues acquire early diagnosis. If the tumor is successfully removed, the symptoms of the disease quickly undergo a reverse development.

Treatment of the androsteromas

Treatment of androsterem only operative - removal of the adrenal tumor affected by a tumor.

Forecast

With early diagnosis and timely surgical treatment, benign androsteroma has a favorable prognosis. With malignant androsteroma and the presence of metastases, the prognosis is unfavorable.