Sex-hormone Producing Tumor
Sex-hormone producing adrenal tumors are rare tumors that make too much androgen (i.e. testosterone), estrogen, or both. These tumors are found in 2 out of every million people and can be either benign (adenomas) or malignant (adrenocortical cancer). Although smaller tumors are more likely to benign, while larger tumors are more likely to be malignant, the only way to determine if a sex-hormone producing tumor is cancer is if there is spread into nearby organs (i.e. local invasion), spread to distant sites like the lung and bone (i.e. metastases), or cancer that comes back (i.e. recurrence).
Signs and Symptoms
Sex-hormone producing tumors can produce either male hormones, female hormones, or both:
Androgen secreting tumors
These tumors make too much testosterone and can cause a virilizing (i.e. masculinizing) syndrome. In females, this can cause deepening of the voice, increased hair growth (i.e. hirsutism), acne, and ambiguous genitalia.
Estrogen secreting tumors
These tumors make too much estrogen and cause a feminizing (i.e. making more like a female) syndrome. In boys, this can lead to developing breasts (i.e. gynecomastia), absence of pubic hair, and delayed growth of the penis. In men, it can cause gynecomastia, infertility, and testicular and penile atrophy.
Mixed sex-hormone secreting tumor
These tumors make too much estrogen and androgen and can cause a mixed picture of signs and symptoms.
Patients suspected of having a sex-hormone producing tumor should have tests aimed at the specific type of tumor. For androgen secreting tumors, high blood testosterone and DHEA-s levels and urine 17-ketosteroids help make the diagnosis. For estrogen secreting tumors, high blood estrogen and urine 17-ketosteroids help make the diagnosis.
After confirming the diagnosis of a sex-hormone secreting tumor, imaging tests will be done to find the location of the tumor(s). Typically a CAT scan or MRI will be done first. These imaging tests can also help find out if there are any ovarian tumors that could be causing the problem. If the CAT scan or MRI does not clearly identify a tumor, a special test called selective venous sampling can be done. In this test, a surgeon or radiologist will draw blood directly from the veins draining both adrenal glands and the ovaries to determine which organ is making too much hormone. Selective venous sampling is usually done as an outpatient procedure.
The only chance of curing someone with a sex-hormone producing tumor is to completely remove the tumor at the time of surgery. Since these tumors are often cancer and have a risk of spreading to lymph nodes, these operations may be done as an open adrenalectomy with lymph node dissection. If the tumor is small, then a minimally invasive approach may be done. (See Adrenal Surgery) In some cases, patients will require additional therapy such as chemotherapy or radiation therapy. In addition, drugs that destroy adrenal tissue such as mitotane or ketoconazole may be used. Young patients with complete removal of the tumor have a better prognosis than older patients with larger tumors.