What is adrenocortical tumor?
Adrenocortical tumor (ACT) is a cancer of the adrenal glands, which are triangle-shaped glands located on both kidneys. These glands produce many chemicals, called hormones. These hormones help the body cope with stress (cortisol), control blood pressure (aldosterone) and keep a proper balance of sodium, potassium and water. Other hormones, known as sex steroids or sex hormones, are produced by a part of the adrenal gland called the adrenal cortex.
The disease is called "functioning ACT" if it causes more hormones to be made than normal, or "nonfunctioning ACT" if it does not. In children, about nine out of 10 adrenocortical tumors are "functioning." In adolescents (teens), only about five out of 10 adrenocortical tumors are "functioning."
In children, many times it is hard to tell the difference between benign (non-cancerous tumors that will not spread) or malignant (cancerous) ACT. A child with either form of ACT should be examined and followed by a team of experts.
How common are adrenocortical tumors?
- ACT is a very rare disease, seen in only about 0.2% of all childhood cancers.
- The disease affects more girls than boys.
- It is most common in children between 1 and 4 years old.
- The tendency to develop adrenocortical tumors may be inherited (passed down in families).
What are the symptoms of adrenocortical tumor?
Symptoms of adrenocortical tumor can include the following:
- A lump in the abdomen (belly)
- Persistent pain in the abdomen or back
- A feeling of "fullness" caused by a tumor pressing against the stomach
For children with functioning ACT, symptoms depend on the amount and type of hormones that are overproduced.
Too many sex hormones may cause early signs of puberty if the hormone being released matches the child’s gender:
- In girls, too much of the female sex hormones (estrogens) may cause early development of breast tissue and (rarely) menstrual periods.
- In boys, too much of the male sex hormones (androgens) may cause increased growth of facial, pubic or underarm hair; more developed sex organs; body odor; acne; and a deep voice.
When a child's gender and the hormone that is overproduced do not match:
- A girl whose tumor produces androgens may have fine hair grow on her face, upper back or arms. Her voice may also become deeper. An older girl may no longer have menstrual periods.
- A boy whose tumor produces estrogens may have growth of breast tissue.
Too much cortisol, a hormone related to stress, may cause the following:
- A fatty, rounded hump high on the back, just below the neck
- "Moon face," a flushed, rounded face with pudgy cheeks
- Weight gain
- Stunted height
- High blood pressure and/or high blood sugars
Too much aldosterone, a hormone important to kidney function, may cause the following:
- Muscle cramps
Tumors may produce more than one group of hormones. Most ACTs in children release androgens, alone or with other hormones.
How is adrenocortical tumor treated?
- Surgery - is usually performed to remove the tumor with the affected adrenal gland (adrenalectomy). Nearby lymph nodes and other tissue may also be removed to find out if the disease has spread and, if it has, to remove it. The type and length of the surgery vary based on the tumor's size and location.
- Chemotherapy ("chemo") - uses powerful medicines to destroy cancer cells or stop them from growing (dividing) and making more cancer cells. The way chemo is given depends on the type and stage of the disease: Chemo may be injected into the bloodstream, so that it can travel throughout the body. Some chemo may be given by mouth.
- Hormonal treatment - is medicines given, usually by mouth, to help relieve the symptoms caused by the extra hormones released by the tumor.
- Radiation therapy - uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing. Radiation therapy is most often used after surgery. The method used will depend on your child’s ACT type, stage and location:
- External radiation uses machines outside the body to deliver the X-ray dose.
- Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into the cancer or close to it.
What are the survival rates for adrenocortical tumors?
- If ACT is caught early and can be removed completely, the five-year survival rate is greater than 85 percent.
- If the cancer has already grown outside the gland, cannot be removed or returns following surgery, the survival rate drops below 40 percent.
Why choose St. Jude for your child’s adrenocortical tumor treatment?
- St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
- St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
- The nurse-to-patient ratio at St. Jude is unmatched—averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
- Researchers from St. Jude and institutions in different parts of the world are working together to learn more about adrenocortical tumors.
- In 1990, St. Jude developed the International Pediatric Adrenocortical Tumor Registry (IPACTR) to provide a central place for data and biological samples, such as small pieces of tumor tissue, to be stored and shared. Investigators use information from this registry to better understand the disease, its risks and outcomes. Clinicians also use data from this registry to design studies and to decide the best treatments.
- Researchers at St. Jude found specific mutations (changes) in the TP53 gene that increase the likelihood that a person will develop ACT.
- St. Jude brings the minds and efforts of doctors and scientists interested in ACT together. St. Jude doctors and scientists from the clinic and the lab regularly meet, in person and online with partners elsewhere. They discuss research and projects and generate new ideas to treat ACT.
- St. Jude takes part in international studies, including work with the Children’s Oncology Group, to find the safest and most effective ways to treat both patients with small tumors and tumors that have spread.
- St. Jude is studying the genetic changes in the tumor and in patients. These studies may reveal new and better ways to treat patients with ACT.
- Complete, quality surgery is an important part of successful ACT treatment. Surgery in children can be hard; adrenal glands can be fragile, and tumors may have clots in the large veins. The expert skills and experience of St. Jude surgeons can help improve patient outcomes.
- St. Jude offers a dedicated team of specialists to meet the needs of children with ACT, including: surgeons; cancer doctors and nurses who treat ACT; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosing ACT; experts in diagnostic imaging and nuclear medicine; genetic counselors; dietitians; child life specialists; psychologists; Quality of Life team members; and scientists.
Associated Clinical Trials
IPACTR: International Pediatric Adrenocortical Tumor Registry
Adrenocortical tumors (carcinoma and adenoma)
- Diagnosis of an adrenocortical tumor (ACT)
- 21 years of age or younger at diagnosis
- Relatives of patients with ACT
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