Tumor of the interstitial tissue of the testicles. Klinefelter’s syndrome
The tumor has a high androgenic activity. In its structure, it can be an adenoma or a carcinoma. Carcinoma metastasizes both in the blood and lymph vessels. A tumor of the interstitial tissue of the testicles occurs in children and adults. In children, it causes premature puberty. The voice is coarse, a beard, mustache appear, abundant growth of pubic hair, in the axillary fossa, on the extensor surfaces of the upper and lower extremities begins. There is an increase in skeleton growth. Skeletal muscles are developing intensively. Penis, gonads, scrotum hypertrophy. The scrotum is pigmented. Often an enlarged sexual gland affected by a tumor is palpated. In adults, against the background of normally developed genital organs, an increase in sexual feeling and potency is observed. But then, due to exhaustion, libido and potency are usually sharply reduced. The content of neutral 17-ketosteroids in the urine is high. The presence of a detailed clinical picture of premature puberty and the frequent presence of a tumor in one of the testicles facilitate the diagnosis. A tumor of the interstitial tissue of the testes should be differentiated with premature puberty in case of damage to the pineal gland. The latter differs from the interstitial testicular tumor in a number of symptoms (nausea, vomiting, sometimes paralysis of the eye muscles and impaired pupil reaction, indicating changes in the area of the sylvian water supply). Early diagnosis, benign tumors and timely surgical intervention are the key to a favorable outcome. The outcome is always unfavorable in the presence of metastases. Treatment. Urgent is the surgical removal of the affected sex gland. Klinefelter’s syndrome Klinefelter’s syndrome is characterized by an increased content of gonadotropins in the blood and urine of the patient. However, despite this, the disease proceeds with a pronounced picture of hypogenitalism. The disease can occur with a constitutional predisposition of the patient. The etiology of the disease is unknown. The disease appears during puberty. Weak, sometimes normal sexual development is observed. There is no vegetation on the face; it is located on the pubis according to the female type in the form of a triangle facing upward. The white line of the abdomen in the area from the navel to the womb is covered with hair. The sex glands are sometimes of normal size, often small. On palpation, the testicles are solid. The crotch is pigmented, there is no hair on it. Aspermia and gynecomastia. The content of follicle-stimulating hormone in the urine is increased, and 17-ketosteroids are reduced or normal. Histologically, during a biopsy of the testicles, it is revealed that the membrane of the seminiferous tubules is hyaline and thickens, germ cells and Sertoli cells are sharply degenerated, and interstitial cells are grouped. Klinefelter’s syndrome should be differentiated with passing age-related gynecomastia. Of decisive importance in the differential diagnosis is the normal content of follicle-stimulating hormone in case of pubertal gynecomastia and elevated in Klinefelter’s syndrome. In unclear cases, a biopsy and sperm test are performed, which greatly facilitates the final diagnosis. Choriogonin is used for treatment. Sometimes it gives good results. 1000-5000 units are administered 2 times a week intramuscularly for three months. If necessary, treatment is repeated. However, choriogonin therapy often leads to moderate gynecomastia. The use of androgenic drugs is highly advisable. Typically, treatment begins with intramuscular administration of testosterone propionate 25 mg 2 times a week for three months. Then the dose is reduced to 20-25 mg per week for another two to four months. Subsequently, the administration of androgens continues sublingually for 10–20 mg per day for several more months. It should be remembered that treatment should be carried out for a long time. However, in order to avoid early closure of the epiphyseal zones, the use of androgens up to 12—13 years of age is contraindicated.