adrenal virilism

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Adrenal Virilism

 

Definition

Adrenal virilism is the development or premature development of male secondary sexual characteristics caused by male sex hormones (androgens) excessively produced by the adrenal gland. This disorder can occur before birth and can lead to sexual abnormalities in newborns. It can also occur in girls and women later in life.

Description

In the normal human body, there are two adrenal glands. They are small structures that lie on top of the kidneys. The adrenal glands produce many hormones that regulate body functions. These hormones include androgens, or male hormones. Androgens are produced in normal girls and women. Sometimes, one or both of the adrenal glands becomes enlarged or overactive, producing more than the usual amount of androgens. The excess androgens create masculine characteristics.

Causes and symptoms

In infants and children, adrenal virilism is usually the result of adrenal gland enlargement that is present at birth. This is called congenital adrenal hyperplasia. The cause is usually a genetic problem that leads to severe enzyme deficiencies. In rare cases, adrenal virilism is caused by an adrenal gland tumor. The tumor can be benign (adrenal adenoma) or cancerous (adrenal carcinoma). Sometimes virilism is caused by a type of tumor on a woman's ovary (arrhenoblastoma).
Newborn girls with adrenal virilism have external sex organs that seem to be a mixture of male and female organs (called female pseudohermaphrodism). Newborn boys with the disorder have enlarged external sex organs, and these organs develop at an abnormally rapid pace.
Children with congenital adrenal hyperplasia begin growing abnormally fast, but they stop growing earlier than normal. Later in childhood, they are typically shorter than normal but have well-developed trunks.
Women with adrenal virilization may develop facial hair. Typically, their menstrual cycles are infrequent or absent. They may also develop a deeper voice, a more prominent Adam's apple, and other masculine signs.

Diagnosis

Endocrinologists, doctors who specialize in the diagnosis and treatment of glandular disorders, have the most expertise to deal with adrenal virilization. Some doctors who treat disorders of the internal organs (internists) and doctors who specialize in treating the reproductive system of women (gynecologists) may also be able to help patients with this disorder.
Diagnosis involves performing many laboratory tests on blood samples from the patient. These tests measure the concentration of different hormones. Different abnormalities of the adrenal gland produce a different pattern of hormonal abnormalities. These tests can also help determine if the problem is adrenal or ovarian. If a tumor is suspected, special x rays may be done to visualize the tumor in the body. Final diagnosis may depend on obtaining a tissue sample from the tumor (biopsy), and examining it under a microscope in order to verify its characteristics.

Treatment

Adrenal virilism caused by adrenal hyperplasia is treated with daily doses of a glucocorticoid. Usually prednisone is the drug of choice, but in infants hydrocortisone is usually given. Laboratory tests are usually needed from time to time to adjust the dosage. Girls with pseudohermaphrodism may require surgery to make their external sex organs appear more normal. If a tumor is causing the disorder, the treatment will depend on the type and location of the tumor. Information about the tumor cell type and the spread of the tumor is used to decide the best kind of treatment for a particular patient. If the tumor is cancerous, the patient will require special treatment depending on how far the cancer has advanced. Treatment can be a combination of surgery, medications used to kill cancer cells (chemotherapy), and x rays or other high energy rays used to kill cancer cells (radiation therapy). Sometimes the doctor must remove the adrenal gland and the surrounding tissues. If the tumor is benign, then surgically removing the tumor may be the best option.

Prognosis

Ongoing glucocorticoid treatment usually controls adrenal virilism in cases of adrenal hyperplasia, but there is no cure. If a cancerous tumor has caused the disorder, patients have a better prognosis if they have an early stage of cancer that is diagnosed quickly and has not spread.

Resources

Periodicals

Willensy, D. "The Endocrine System." AmericanHealth April 1996: 92-3.

Key terms

Glucocorticoid — A hormone produced by the adrenal gland; this hormone leads to an increase in blood sugar and creation of sugar molecules by the liver.
Hydrocortisone — A hormone in the group of glucocorticoid hormones.
Prednisone — A drug that functions as a glucocorticoid hormone.

a·dre·nal vir·i·lism

virilism produced by excessive or abnormal secretory patterns of adrenocortical steroids.

adrenal virilism

a condition characterized by hypersecretion of adrenal androgens, resulting in somatic masculinization. Excessive production of the hormone may be caused by a virilizing adrenal tumor, congenital adrenal hyperplasia, or an inborn deficiency of enzymes required to transform endogenous androgenic steroids to glucocorticoids. Girls born with adrenogenitalism may be pseudohermaphroditic with clitoral enlargement and labial fusion in infancy and later have low vocal pitch, acne, amenorrhea, and masculine distribution of hair and muscle development. Boys with congenital adrenogenitalism show precocious development of the penis, the prostate, and pubic and axillary hair, but their testes remain small and immature because negative feedback from the high level of adrenal androgens prevents the normal pubertal increase in pituitary gonadotropin levels. Children with the disorder are unusually tall, but their epiphyses close prematurely, and as adults they are abnormally short. Virilizing tumors are more common or more frequently diagnosed in women; they usually occur between 30 and 40 years of age but may arise later, after menopause. Signs of the tumor in women include hirsutism, amenorrhea, oily skin, ovarian changes, muscular hypertrophy, and atrophy of the uterus and breasts. Treatment may involve tumor resection, cortisol administration, and cosmetic surgery. Electrolytic hair removal may be indicated. Also called adrenogenital syndrome.

adrenal virilism

The appearance of male characteristics in a female as a result of excessive production of male sex hormones by the adrenal gland. This may cause a form of PSEUDOHERMAPHRODITISM.

virilism

the presence of male characteristics in the female. See also virilization.

adrenal virilism
virilism caused by a tumor of the zona reticularis of the adrenal gland.