ACC is rare, the cause is unknown. Smoking may increase the risk of ACC. It occurs more often in women than men. It can happen at any age but the typical patient is about 50 years old. About 50% of these tumors are functional and 50% are non-functional. A variety of hormones can be produced by functioning tumors, most commonly an excess of sex hormones called androgens. The male hormone (testosterone) and the female hormone (estrogen) are common examples of androgens. If too much testosterone is produced in a female with ACC, the patient may notice and increase in body hair,a deepening voice, increase sex drive or thinning hair. In a male these changes may be subtle. If to much estrogen is made in a male with ACC, the patient may notice loss of muscle mass, enlargement of the breast or impotence. Likewise, in a female these symptoms may be subtle. Less commonly, ACC can produce too much cortisol leading to Cushing’s syndrome or too much aldosterone leading to Conn’s syndrome. Blood test are done to detect these changes and then a CAT scan or an MRI can be done to see the tumor. Diagnosis as cancer may be difficult. Size is the best predictor. Tumors larger than 6-8 cm are more likely cancer. Tumors that produce male or female hormones are more likely to be cancer. Surprisingly, a biopsy of the mass does not settle the issue and should not be done because of risk implanting cancer cells along the biopsy tract and because it will not reliably diagnose or exclude cancer.
A functioning ACC usually is detected earlier than non-functioning ACC and may be smaller and therefore more effectively treated. Non-functioning ACC usually presents with local symptoms such as flank discomfort or palpable mass or may be found on an imaging study done for an unrelated reason.
ACC is an aggressive tumor. Surgical resection to a negative margin is the mainstay of treatment. With small tumors ( <5 cm) this may be done with laparoscopic techniques. However, most ACC are relatively locally advanced at the time of diagnosis and larger than 6 cm and open techniques are required to remove it along with a rim of uninvolved tissue. ACC commonly invades other tissues in the area such as the kidney, liver or diaphragm. Portions of these structures may need to be removed to remove all the tumor. Other therapies such as chemotherapy (Mitotane) and radiation therapy may be helpful after the surgery, but removal of the tumor with a margin of normal tissue is the critical aspect of therapy. The aggressiveness of the tumor, it’s relative resistance to chemotherapy and radiation therapy, and the fact that it is commonly diagnosed relatively late, make ACC difficult to cure. Recent studies with antiangiogenesis agents and a class of drugs call tyrosine kinase inhibitors has show promise. Re-growth of the tumor( local recurrence) and metastatic disease commonly to the lungs and liver are problems after initial surgery.