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.
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.
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.
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.