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About Adrenocortical Carcinoma

  • At a Glance

    • Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland.
    • Having certain genetic conditions increases the risk of adrenocortical carcinoma.
    • Symptoms of adrenocortical carcinoma include pain in the abdomen.

    What Is Adrenocortical Carcinoma?

    Adrenocortical carcinoma is a rare disease in which malignant (cancer) cells form in the outer layer of the adrenal gland. There are two adrenal glands; they are both small and shaped like a triangle. One adrenal gland sits on top of each kidney. Each adrenal gland has two parts. The outer layer of the adrenal gland is the adrenal cortex. The center of the adrenal gland is the adrenal medulla.


  • illustration of adrenal glands

    There are two adrenal glands, one on top of each kidney. The outer part of each gland is the adrenal cortex; the inner part is the adrenal medulla.


  • The adrenal cortex makes important hormones that:

    • Balance the water and salt in the body.
    • Help keep blood pressure normal.
    • Help control the body's use of protein, fat, and carbohydrates.
    • Cause the body to have masculine or feminine characteristics.

    Adrenocortical carcinoma is also called cancer of the adrenal cortex. A tumor of the adrenal cortex may be functioning (makes more hormones than normal) or nonfunctioning (does not make more hormones than normal). Most adrenocortical tumors are functioning. The hormones made by functioning tumors may cause certain signs or symptoms of disease, as described below.

    The adrenal medulla makes hormones that help the body react to stress. Cancer that forms in the adrenal medulla is called pheochromocytoma.

    Adrenocortical carcinoma and pheochromocytoma can occur in both adults and children. Treatment for children, however, is different from treatment for adults.

    Risk Factors

    Having certain genetic conditions increases the risk of adrenocortical carcinoma (risk factors). Having a risk factor does not mean that you will get cancer, and not having risk factors doesn't mean that you will not get cancer. Our physicians will review your risk factors with you.

    Risk factors for adrenocortical carcinoma include having the following hereditary conditions:

    Signs and Symptoms

    Signs and symptoms of adrenocortical carcinoma include:

    • A lump in the abdomen
    • Pain in the abdomen or back
    • A feeling of fullness in the abdomen

    A nonfunctioning adrenocortical tumor may not cause signs or symptoms in the early stages.

    A functioning adrenocortical tumor makes too much of one of the following hormones:

    • Cortisol
    • Aldosterone
    • Testosterone
    • Estrogen

    Too much cortisol may cause:

    • Weight gain in the face, neck, and trunk of the body, and thin arms and legs
    • Growth of fine hair on the face, upper back, or arms
    • A round, red, full face
    • A lump of fat on the back of the neck
    • A deepening of the voice and swelling of the sex organs or breasts in both males and females
    • Muscle weakness
    • High blood sugar
    • High blood pressure

    Too much aldosterone may cause:

    • High blood pressure
    • Muscle weakness or cramps
    • Frequent urination
    • Feeling thirsty

    Too much testosterone (in women) may cause:

    • Growth of fine hair on the face, upper back, or arms
    • Acne
    • Balding
    • A deepening of the voice
    • No menstrual periods

    Men who make too much testosterone do not usually have signs or symptoms.

    Too much estrogen (in women) may cause:

    • Irregular menstrual periods in women who have not gone through menopause
    • Vaginal bleeding in women who have gone through menopause
    • Weight gain

    Too much estrogen (in men) may cause:

    • Growth of breast tissue
    • Lower sex drive
    • Impotence

    These and other signs and symptoms may be caused by adrenocortical carcinoma or by other conditions. Check with your doctor if you have any of these problems.

    Diagnostic Tests

    Tests and exams used to diagnose adrenocortical carcinoma include:

    • Physical exam
    • Biopsy
    • 24-hour urine test
    • Blood chemistry study
    • CT scan or MRI

    Learn more about how we diagnose adrenocortical carcinoma.

    Treatments

    There are different types of treatment for patients with adrenocortical carcinoma. Treatments include:

    • Surgery
    • Radiation therapy
    • Chemotherapy
    • IR-guided ablation

    Learn more about how we treat adrenocortical carcinoma.

    Factors Affecting Treatment Options and Recovery

    Prognosis (chance of recovery) and treatment options depend on:

    • The stage of the cancer (the size of the tumor and whether it is in the adrenal gland only or has spread to other places in the body).
    • Whether the tumor can be completely removed in surgery.
    • Whether the cancer has been treated in the past.
    • Your general health.
    • The grade of tumor cells (how different they look from normal cells under a microscope).

    Adrenocortical carcinoma may be cured if treated at an early stage.