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About Childhood Adrenocortical Carcinoma

  • 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 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, may cause patients to develop masculine traits regardless of gender.
    • This type of cancer can spread to the kidneys, lungs, bones, and brain.
    • Many patients often have Li-Fraumeni syndrome, an inherited condition that predisposes them to many cancer types.

    At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, we bring together experts in pediatric oncology, endocrinology, and surgery. The combined expertise offers children with adrenocortical carcinoma access to top pediatric cancer care in the nation. Doctors in our Rare Tumors Program care for children with adrenocortical carcinoma.

    Symptoms of Childhood Adrenocortical Carcinoma

    The most common symptoms of adrenocortical carcinoma include:

    • Palpable abdominal mass
    • 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

    How We Diagnose Childhood Adrenocortical Carcinoma

    Our doctors take the first step in treating your child by ordering diagnostic tests to form an accurate diagnosis. Testing may include imaging studies, blood and urine tests, and a bone scan.

    After the medical team completes all tests, your child’s doctor will discuss the most effective treatment options with your family.

    How We Treat Childhood Adrenocortical Carcinoma

    We use the latest and most advanced treatment options to provide your child with the best possible outcome.

    Treatment may include:

    • Surgery: Surgery may involve removing the entire tumor and nearby tissue and possible removal of the adrenal gland. We use a minimally invasive approach, or laparoscopy, whenever possible.
    • Chemotherapy: We may use chemotherapy, a drug treatment that aims to destroy or shrink cancer cells, to manage a disease that has spread.

      Different groups of chemotherapy drugs work in different ways. Often, we will use a combination of chemotherapy drugs. Your child may receive chemotherapy:

      • Orally, as a pill to swallow
      • Intramuscularly, as an injection into the muscle or fat tissue
      • Intravenously, as a direct injection into the bloodstream or IV
      • Intrathecally, as a direct injection into the spinal column through a needle

      While chemotherapy effectively treats certain cancers, the drugs cannot differentiate normal healthy cells from cancer cells. As a result, there can be adverse side effects during treatment. Being able to anticipate these side effects can help the care team, child, and family prepare and, in some cases, prevent these complications from occurring.

    • Radiation therapy: This treatment uses high-energy rays from a specialized machine to damage or destroy cancer cells and shrink tumors. We will use radiation therapy for tumors that are unresponsive to surgery and radiation.
    • Hormone therapy: We may use medication that inhibits the masculinizing effects of the tumor.

    Beyond treatment, we continue to care for children with adrenocortical carcinoma through our pediatric cancer survivorship programs. We see children regularly to manage disease complications, screen for early recurrence of cancer, and control late effects of treatment. We also provide comprehensive counseling and support groups.

    Long-term Outcomes for Children with Adrenocortical Carcinoma

    When a child’s tumor is detected early, the survival rate is close to 90 percent. Complete surgical removal offers the greatest hope for a cure.

    In the case of advanced tumors, the survival rate is lower. The overall survival rate is 54 percent.

    Childhood Adrenocortical Carcinoma Treatment Team

    We bring together an expert team of pediatric specialists to treat children with adrenocortical carcinoma. View a complete list of the specialists in our Childhood Solid Tumor Center.