• Adrenocortical Carcinoma Overview

    Adrenocortical carcinoma is a rare cancer that forms in the outer layer of the adrenal glands. These glands, which are near the front side of the kidneys, produce hormones such as different forms of steroids. These hormones control several body functions, including blood pressure, response to stress, and sexual development.

    Adrenocortical carcinoma typically affects children younger than 4 and older than 13. These tumors, which frequently secrete hormones and may cause patients to develop masculine traits (regardless of gender), can spread to the kidneys, lungs, bones and brain. Many patients often have Li-Fraumeni syndrome, an inherited condition that predisposes them to many types of cancer.

    Adrenocortical Carcinoma Treatment at Dana-Farber/Boston Children’s

    Children and adolescents with adrenocortical carcinoma are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Solid Tumor Center. Continue reading to learn more about adrenocortical carcinoma or visit the Rare Tumors Treatment Program homepage to learn about our expertise and treatment options.

    Symptoms & Diagnosis

    The most common symptoms of adrenocortical carcinoma include a palpable abdominal mass and adrenocortical hormone overproduction. In boys this hormonal overproduction can cause:

    • Premature puberty with enlargement of the penis and scrotum
    • Premature growth and increase in muscle mass
    • Pubic hair
    • Acne
    • Deepening voice

    In girls, it can cause:

    • Premature appearance of pubic and underarm hair
    • Clitoral enlargement
    • Acne
    • Deepening voice
    • Premature increase in growth
    • Lack of appropriate breast development
    • Delayed menstrual cycle
    • About 10 percent of patients have signs of Cushing syndrome, including a round face, double chin, generalized obesity, growth failure and hypertension.

    In addition to a complete medical history and physical examination, adrenocortical carcinoma is diagnosed with:

    • Blood and urine tests
    • Computerized (CT or CAT) scan
    • Magnetic resonance imaging (MRI)
    • Ultrasound
    • Bone scan to detect disease that has spread to the bones 

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care

    Adrenocortical carcinoma is treated with:

    • Surgery—the mainstay of treatment and currently the major hope for a cure
    • Chemotherapy—for disease that has spread and cannot be surgically removed or if evidence of a tumor persists following surgery
    • Radiation therapy—for tumors that cannot be surgically removed and do not respond to chemotherapy

    Additional treatment may include artificial hormones that block the masculinizing effects of the tumor.

    If recurrence occurs, adequate surgical removal is associated with improved survival compared to patients whose tumors cannot be surgically removed.

    Long-term Outlook

    As surgical techniques improve, so too should prognosis. The survival rate for patients whose tumors are detected at an early stage approaches 90 percent; however, survival decreases with more advanced tumors. The overall survival rate is 54%. Complete surgical removal can cure adrenocortical carcinoma.