One of the less common types of cancer is adrenal cortical (or adrenocortical) carcinoma, abbreviated as ACC. Dr. Irina Veytsman, Dr. Lynnette Neiman, and Dr. Tito Fojo of the National Cancer Institute wrote a review of its characteristics and treatment options. The survival rate at 5 years is low, in part because it is usually diagnosed at an advanced stage. Better awareness of this unfamiliar cancer may help improve the statistics.
ACC may have no symptoms, or it may increase production of adrenal hormones, including cortisol. Excess cortisol in turn produces Cushing's syndrome, with a characteristic “moon face” appearance. Acne, decreased growth in children, high blood pressure, potassium deficiency, and weight gain are other possible symptoms of ACC related to cortisol production. In some patients, estrogen or androgen production is also increased, leading to feminine features in males or masculine features in females.
ACC is slightly more common in women than in men. It has a bimodal age distribution, affecting preferentially children younger than 5 years or adults in their 40's and 50's. The cause is unknown.
The primary treatment is surgery, which can cure some cases completely with one operation. Recurrence is common, however. Veytsman and coauthors reported they believe repeat surgery may improve survival, but the benefits are difficult to quantify.
The drug mitotane is used for ACC and other causes of Cushing's syndrome. Mitotane is an isomer of the insecticide DDE, which is chemically similar to the more infamous insecticide DDT. It is highly effective at managing hormone excess, but has side effects that are intolerable for many. These include gastrointestinal and neurological symptoms.
A Swedish group with first author Dr. B. Wangberg reported on a series of 43 patients with ACC treated in the same surgical center. All were offered mitotane treatment, unless other illnesses ruled this out. Most of these patients were able to tolerate high-dose mitotane, which increased the 5-year survival rate from 28.6 percent to 64.0 percent in patients with advanced cancer.