Androgen tumor of the adrenal cortex
Endocrinology / / April 24, 2016
hormonally active tumors of the adrenal cortex can produce in excess cortisol or aldosterone, and androgens.In the first case developed Cushing's syndrome - Cushing, in the second - primary aldosteronism (Conn's syndrome).By androgen-producing tumors of the adrenal cortex are those tumors that secrete increased amounts in the mainly or exclusively steroids having androgenic activity.
Androsteromy - tumors that produce androgens, can be benign or malignant.Benign androsteromy built mostly of dark cells, sometimes forming tyazhistye structures resembling the reticular zone of the adrenal cortex.The cells and their nuclei differ large polymorphism.Most hyperchromatic nuclei, but there are large bright nucleus.Lipids in the tumor tissue is small, there are lumps of lime.
Malignant androsteromy constructed of cellular elements that are close to those that make up the corresponding benign tumor, but they differ a great polymorphism of nuclei, a large number of polynuclear giant cells, the pres
When virilizing tumors of the adrenal cortex is determined by the specific action of hormonal steroids having hormonal activity, which in excessive amounts are produced by cells of the tumor.
With the development of tumors in childhood in girls enjoying rapid growth and accelerated maturation of skeletal muscle development, the appearance of acne, the vegetation on the body, deepening of voice, clitoral hypertrophy.In boys, there is a picture of precocious puberty on izoseksualnomu type, but the testicles are atrophic.As boys and girls alike accelerated physical development and maturation of the skeleton.The mental development of the child usually corresponds to his true age.
Women in the development of androgen-producing tumors of the adrenal cortex observed defeminizatsiya - menstruation is rarely done and scarce, and then comes amenorrhea, mammary gland atrophy.Uterine size decreases, the clitoris hypertrophy.Along with this, there are male secondary sexual characteristics - vegetation on the face and body, acne on his face;may be a balding head male pattern.The voice is low.Muscle hypertrophy, muscle strength increased.Facial features grubeyut, omuzhestvlyayutsya.Due to muscle development and redistribution of fat deposits in the subcutaneous tissue, as well as due to the atrophy of the breast contours of the body change - body approaches the male.
In adult males the clinical signs of androgen-producing tumors of the adrenal cortex is difficult trapped.In some cases, these tumors in men are disguised as hormonally inactive adrenal tumors and maybe even under the guise of incorrectly interpreted kidney tumors.
Because tumors in the adrenal cortex, predominantly produce androgens, often occurs to a greater or lesser extent, increase the production of other adrenocortical hormones, in some patients, along with virilization, there are other signs of Cushing.
When hormonal study, the most characteristic feature of androgen-producing tumors of the adrenal cortex is increased release of urine 17-keto steroids, sometimes modest - up to 50-60 mg / 24 hours, sometimes - very large - up to several hundredand even up to 1000 mg / 24 hr. fractionation 17-keto steroids can be isolated note allocation increase beta fraction having adrenal origin.Of particular importance is the increase in the production of one of the ketosteroidov - dehydroepiandrosterone, which is particularly characteristic of androgen-producing tumors of the adrenal cortex.
addition, in cases where the tumor cells produce in excess not only steroids having androgenic activity and, in varying degrees and other corticosteroids can be a high blood and excretion of urine 17 gidroksikortikosteroidov particularlycortisol.
Some patients have elevated urinary excretion of estrogen.Given that estrogens are produced by the adrenal glands in the same cells as the 17-keto steroids and production of the adrenal glands is increased by stimulation of the adrenal adrenocorticotropic hormone, it can be assumed that the increased release of estrogen in these patients is associated with increased their production of tumor cells.
In some cases, significantly increased urinary excretion pregnandiol.
Diagnosis and differential diagnosis
diagnosis of androgen-producing tumors of the adrenal cortex is based primarily on the characteristic clinical picture - signs of virilization in girls and women who did not have prior to the beginning of the disease disorders of the reproductive system, and premature sexual development inboys, accompanied by atrophy of the testes.The most significant sign of the laboratory androgen-producing tumors of the adrenal cortex is to increase the allocation of urine 17-keto steroids and especially dehydroepiandrosterone.
considerable importance in the diagnosis of tumors of the adrenal cortex are functional assays, such as Cortisone or prednisolone.Tumors of the adrenal cortex usually have a certain "autonomy" - their function is not under regulatory influence of pituitary adrenocorticotropic hormone.Consequently, suppression of adrenocorticotropic pituitary function glucocorticoid does not reduce the steroid production by tumor cells and isolating the 17-keto steroids in urine after administration of glucocorticoid to the patient is not significantly reduced.It should be noted that the literature contains some descriptions when secretion of corticosteroids in patients with benign and malignant tumors was even a certain regulation, depending on the pituitary.
the differential diagnosis of tumors of the adrenal cortex may be used and the sample with the introduction of ACTH.Patients with tumors after ACTH administration of 17-ketosteroids allocation is not changed, while in patients with adrenocortical hyperplasia increases.
important diagnostic method that allows not only to establish the presence of a tumor of the adrenal cortex, but also to specify its location, is the X-ray.X-rays kidneys and adrenal glands are made after the introduction of gas into the tissue surrounding the organs.Gas can be entered directly into the perirenal fat and retroperitoneal fat in Presacral puncture.Last gas injection method is preferable, since it falls in the gas region of both kidneys.Against the backdrop of the gas injected konturiruyutsya kidneys and adrenal glands and the tumor can be detected.
effectiveness of the method is increased when used in addition to the usual review of tomograms images.
This study is useful to combine with excretory urography or retrograde pyelography, reveals some indirect signs of adrenal tumors, in particular, the displacement or deformation of the kidney.
This method of research is safe.When used as a contoured gas than air, and carbon dioxide or oxygen and introducing it through Presacral puncture hazard of air embolism is practically absent.
However, with the help of X-ray study fails reliably establish the presence of only relatively large tumors, and, according to some authors, the incidence of tumors using oksisuprarentgenografii does not exceed 50%.Indirect evidence of a tumor, if it is not contoured, can be obtained on the basis of the displacement detection kidney.
These methods of research focused mainly on the differential diagnosis of tumors of the adrenal cortex and other virility syndromes associated with adrenal gland disorders, particularly congenital virilizing adrenal hyperplasia.Along with this, virilizing tumor of the adrenal cortex must be differentiated with virilization associated with gonads disease.
In patients with androgen-producing tumors of the ovaries - arrhenoblastoma (or androblastoma) lipidokletochnymi tumors can develop virility syndrome, clinically difficult to distinguish from the adreno-genital syndrome, in particular from the syndrome that develops in patients with virilizing tumors of the adrenal cortex.The differential diagnosis of these diseases is based on data from physical examination - detection of ovarian cancer at the gynecological examination or a tumor of the adrenal gland when oksisuprarentgenografii, as well as on the data of hormonal research.When ovarian arenoblastomah allocation of 17-keto steroids can be normal or moderately elevated;increasing isolation of 17-ketosteroids with no degree corresponds virilization.Perhaps this is due to the fact that virilization in patients arenoblastomoy has less to do with the development of tumor cells, 17-keto steroids, as with testosterone, detected in the tumor tissue.Increasing the amount of testosterone has little effect on the allocation of 17-keto steroids.
- Physiology of the adrenal cortex
- Chronic adrenal insufficiency.Addison's disease
- blood supply and innervation of the adrenal
- Clinical evaluation of laboratory studies of the functional state of the adrenal cortex
- Determination of corticosteroids and their metabolites in urine
- Diagnosis and differential diagnosis of congenital adrenal hyperplasia
- Treatment of chronic adrenal insufficiency
- apoplexy adrenal cortex.Syndrome Waterhouse-Friderichsen (waterhouse- friderichsen)
- virilizing congenital adrenal hyperplasia
- Treatment of congenital adrenal hyperplasia
- Acute adrenocortical insufficiency
certain value can be fractionation 17-keto steroids.When tumors of the adrenal cortex is usually marked by a significant increase in allocation of dehydroepiandrosterone, when arenoblastomah increased mainly androsterone isolation and to a lesser extent etioholanolona.
Patients lipidokletochnymi virilizing ovarian tumors release of 17-keto steroids can be either normal or increased significantly, and such a study can not serve the purposes of differential diagnosis of this rare disease and virilizing tumors of the adrenal cortex.
When sclerocystic ovaries virilization is rarely so severe that there was a need in the differential diagnosis between this disease and virilizing tumor of the adrenal cortex.
must be borne in mind that sclerocystic ovaries may develop in patients virilism adrenal origin.Total excretion of 17-ketosteroids patients sclerocystic ovaries usually normal or slightly above normal (as opposed to patients with tumors of the adrenal cortex).When fractional division 17-keto steroids in patients with ovarian sclerocystic notes the increasing isolation and etioholanolona androsterone, dehydroepiandrosterone and not as in patients with virilizing tumors of the adrenal cortex.
The boys need to carry out a differential diagnosis between precocious puberty associated with the pathology of the central nervous system, in particular in the area of the pineal gland tumors, and androgen-producing tumors of the adrenal glands.When precocious puberty central genesis in parallel with other signs omuzhestvleniya holds testicular growth, the emergence of spermatogenesis.This form of "true" precocious puberty observed in some patients with congenital virilizing adrenal hyperplasia, but tumors of the adrenal cortex with a significant development of the external genitalia is usually atrophic testes.Isolation of 17-keto steroids in boys with precocious puberty central origin can be raised to a level corresponding to their isolation from adult males, but does not reach a high degree of inherent in patients with tumors of the adrenal cortex.What matters is the identification of the pathological process in the brain - the presence of symptoms of increased intracranial pressure, focal nervous symptoms, etc., which may be the cause of precocious puberty...Differential diagnosis
precocious puberty in connection with testicular tumor is detected based on the latter palpable tumor.Increased allocation of 17-keto steroids is less than the adrenal tumor testicular tumor.
Treatment virilizing adrenocortical tumors surgically.In the case where tumor cells are selectively produce in excess only steroids having androgenic, glucocorticoid and products normal braking adrenocorticotropic pituitary function and associated second adrenal atrophy does not occur.In this case there is no danger hypocorticoidism in the postoperative period.However, both clinically and hormonal examination is not always possible to exclude patients with virilizing tumors of the adrenal cortex certain degree of increase cortisol production.Therefore safer surgery for virilizing tumors of the adrenal cortex to carry out during the treatment cortisol, as is recommended in operations for tumors of the adrenal cortex that occur with Cushing's syndrome Itsenko-.Dose steroids thus may be somewhat smaller.After surgery, corticosteroid dose is gradually reduced.Drug withdrawal speed determined by the presence or absence of symptoms adrenocortical insufficiency.