Adrenocortical Tumors

Also called: ACC, adrenocortical cancer, adrenocortical tumor, cancer of the adrenal cortex, adrenocortical adenoma, adrenocortical carcinoma and adrenocortical cancer

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. The hormones help the body cope with stress, control blood pressure and keep a proper balance of sodium, potassium and water.

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 carcinoma (cancerous tumors that will spread) and adenoma (non-cancerous tumors that will not spread). All children with adrenocortical tumor (carcinoma or adenoma) should be carefully assessed and followed by a team of experts. Some adenomas can develop into cancerous tumors.

How common are adrenocortical tumors?

  • ACT is a very rare disease, seen in only about 0.2 percent 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)
  • Pain in the abdomen or back
  • A feeling of fullness

For children with functioning ACT, symptoms depend on the amount and type of hormones that are overproduced.

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 related to kidney function, may cause the following:

  • Muscle cramps
  • Weakness
  • Thirst

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. ACT that produces only estrogens is very rare.
  • 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.

Tumors may produce more than one group of hormones. Most ACT in children release androgens, alone or with other hormones.

How is adrenocortical tumor treated?

  • Chemotherapy (“chemo”) - uses powerful medicines to kill 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 tumor releasing too many hormones. These medicines can help patients feel better.
  • 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 to provide a central place for data and tumor samples 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. For example, St. Jude scientists created an adrenal tumor model in the lab that scientists use to test drugs for ACT. St. Jude doctors and scientists from the clinic and the lab regularly meet together, in person and online with partners elsewhere, to discuss the best new ideas for ACT.
  • As a result of research conducted at St. Jude, the Children’s Oncology Group created a treatment program for many institutions that studies the biology of the tumors and how often different types of TP53 mutations occur.
  • 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 actively studying the gene changes in the tumor and in patients, which may help scientists find new and better ways to treat patients with ACT.
  • Complete, quality surgery is an important part of treating ACT successfully. 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 (pathologists); experts in diagnostic imaging and nuclear medicine; genetic counselors; dietitians; child life specialists; psychologists; Quality of Life team members; researchers; scientists, and many others.

Associated Clinical Trials

IPACTR: International Pediatric Adrenocortical Tumor Registry

Diseases Treated:

Adrenocortical carcinoma

Eligibility:

  • Diagnosis of an adrenocortical tumor (ACT)
  • 21 years of age or younger
  • Relatives of patients with ACT
View Trial

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