ANDROSTEROMA (Greek anzr, andros — the man, stereos — firm + - Uma; synonym adrenosteroma) — the gormonalnoaktivny tumor of bark of adrenal glands producing male sex hormones (androgens).
And. it is localized in one of adrenal glands or in the locations of additional adrenal fabric — ovaries, a wide ligament of a uterus (8 — 10% of observations).
And. occurs usually at persons of young age (up to 35 — 40 years). At girls the tumor is observed by 5 times more often than at boys. Almost in 60% of cases of a tumor malignant also metastasize, as a rule, in retroperitoneal limf, nodes, a liver and lungs. At children in 4% of observations of a tumor were regarded as inborn — at the birth signs are noted virilescences (see). Especially high percent of malignant tumors occurs at children, and the child is younger, the more often the new growth has malignant character.
Both high-quality, and malignant And. are put into the connective tissue capsule of a round or oval form. Weight And. fluctuates from 5 g to 2 kg, and hormonal activity not always depends on the size of a tumor and duration of its existence.
The microscopic picture is in most cases presented by accumulation of preferential dark cells which on structure and an arrangement remind a structure of a mesh zone of bark of adrenal glands. In malignant And. the cellular atipizm, polymorphism, existence of multiple mitoses, an invasion of tumor cells in vessels and the capsule is to some extent expressed.
The symptomatology is caused by secretion by a tumor androgens (see). The hormonal activity of a new growth is higher, the signs of virilescence are expressed stronger. Metastasises as well as primary tumor, have hormonal activity, i.e. ability to allocate androgens. The picture of a disease at women is characterized by frustration of a menstrual cycle (an amenorrhea or an oligomenorrhea), a hypertrophy of a clitoris, pilosis of the person and body, a masculinization of a figure (fig.), coarsening of a voice, sometimes baldness of the head on men's type. At certain patients the hypertension and disorders of carbohydrate metabolism in the form of a hyperglycemia and moderately expressed glucosuria can be observed.
At men And. meet extremely seldom and are not shown by any external signs therefore the diagnosis at them is established in a late stage of a disease when the tumor reaches the considerable sizes and is available to a palpation.
And. at children it is characterized by a picture of premature sexual and physical development: isosexual at boys, heterosexual at girls. They usually considerably advance in growth of the peers and differ in a brawny constitution. Early ossification of long tubular bones and premature closing of regions of growth is observed, i.e. the bone age considerably advances actual. Premature sexual development cannot be considered true since it is expressed, as a rule, only in emergence of secondary sexual characteristics (pilosis of external genitals, persons, sometimes a trunk and extremities, coarsening of a voice, increase in a clitoris or penis) while gonads (testicles, ovaries) usually correspond to age of the child. However single observations when at boys with are described And. at a research of testicles found a spermatogenesis.
Content in daily urine 17 of ketosteroids (metabolites of androgens) at patients And. it is almost always raised and sometimes by 5 — 50 times exceeds normal indicators, generally at the expense of fractions of a degidroepiandrosteron and etiocholanolone. Especially high level of allocation of 17 ketosteroids is noted at patients with malignant tumors. Excretion with urine of intermediate products of synthesis of androgens, and in particular pregnandiol, can also be high.
the Diagnosis is made on the basis of clinical symptoms and datas of laboratory. The maintenance of a high caption of androgens and their metabolites helps with daily urine to establishment of the diagnosis. Make a tomography of adrenal glands in the conditions of a retropneumoperitoneum for establishment of the topical diagnosis. In 95% of cases it is possible to establish localization of a tumor. For the specified purposes instead of a retropneumoperitoneum the angiography of adrenal glands can be used. However interpretation of the received angiograms sometimes presents considerable difficulties, and the percent of wrong diagnosis in these cases is higher, than at use of a retropneumoperitoneum. At the expressed clinical picture A. and absence of a tumor in adrenal glands make a pneumoperitoneum for the purpose of detection of the new growth which developed from malrelated adrenal tissue; on a ginekogramma increase in one of ovaries can be revealed. If localization of a tumor is unknown, it is reasonable to make a laparotomy that helps to examine at the same time both adrenal glands and bodies of a small pelvis (the place of a possible arrangement malrelated And.).
Differential diagnosis it is carried out with an inborn hyperplasia of bark of adrenal glands (see. Adrenogenital syndrome ), a syndrome of Matte — Leventalya (see. Matte — Leventalya a syndrome ), arrhenoblastoma (see), a tumor of testicles at boys. For differential diagnosis between And. and an adrenogenital syndrome use tests with loading glucocorticoids (dexamethasone, a hydrocortisone, Prednisolonum). Falloff of allocation of 17 ketosteroids after introduction of one of the specified drugs is observed at a hyperplasia of bark of adrenal glands while at patients And. content in urine 17 of ketosteroids does not change (a dosage and a technique of conducting test — see. Dexamethasonal test ). At patients with a syndrome of Shteyna-Leventalya, an arrhenoblastoma and a tumor of testicles the content in daily urine 17 of ketosteroids corresponds to usually normal indicators or is a little increased that helps with establishment of the differential diagnosis.
Timely performed operation leads the forecast to a favorable outcome not only at high-quality And., but also at considerable number of patients at malignant A. Proiskhodit normalization of exchange processes and appearance of patients. At children the growth inhibition because of early ossification of epiphyseal cartilages can be observed further. And still patients with malignant tumors shall have a careful forecast.
Treatment surgical. At an arrangement of a tumor in one of adrenal glands for removal And. make a lumbotomy. At localization And. in the right adrenal gland operation should be made with extra care because of direct contact of a tumor with the lower vena cava.
Unlike patients kortikosteromy, after removal And. it is possible not to administer the corticosteroid drugs since the adrenal gland opposite to a tumor is not atrophied.
Bibliography: Zhukovsky M. A. Children's endocrinology, page 131, M., 1971; Nikolaev O. V. and Kertsman V. I. Kortikosteroma, M., 1970; Nikolaev O. V. and E. I Cockroaches. Hormonal and active tumors of bark of an adrenal gland, page 91, M., 1963; Soffer L., Dorfman R. and Gebrilav of L. Epinephral glands of the person, the lane with English, M., 1966.
V. I. Kertsman.