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Adrenocortical Tumor Treatment

Also called: ACT, adrenocortical cancer, cancer of the adrenal cortex, adrenocortical adenoma, adrenocortical carcinoma

Adrenocortical tumor (ACT) is a cancer of the adrenal glands. There are 2 types of ACT:

  • Functioning ACT causes more hormones to be made than normal.
  • Nonfunctioning ACT does not.

In children, about 90% (9 in 10) of ACT is the functioning type.  In teens, only about 50% (1 in 2) of ACT is the functioning type.

ACT occurs in about 0.2% of all childhood cancers. It affects more girls than boys. It is most common in children between 1–4 years old.

If ACT is caught early and can be removed completely, the 5-year survival rate is over 85%. If the cancer has already grown outside the gland, cannot be removed, or returns following surgery, the survival rate is under 40% (4 in 10).

Find out more about ACT on the Together by St. Jude™ online resource.

Treatment of adrenocortical tumor

An ACT is often treated with surgery, followed by chemotherapy, hormonal treatment, and radiation therapy.

  • 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 and making more cancer cells. 
  • Hormonal treatment involves 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 of the cancer, and location:

Adrenocortical tumor clinical trials

St. Jude offers clinical trials and cancer research studies for children, teens, and young adults for adrenocortical tumor. Learn more about clinical research at St. Jude.

Recruiting
3CAR: B7-H3-specific CAR T-cell Therapy for Children and Young Adults with Solid Tumors

Study goal:

The main purpose of 3CAR is to find out if this type of immunotherapy is safe for pediatric patients with solid tumors. We also want to learn if it is effective in fighting solid tumors.

Age:

Up to 21 years old

Recruiting
IPACTR: International Pediatric Adrenocortical Tumor Registry

Study goal:

The main goal of IPACTR is to collect relevant clinical and biological data, including blood and tissue samples, to help researchers better understand the 1) common signs and symptoms of ACT, 2) length of time between the first signs and symptoms and diagnosis of ACT, 3) how genetic conditions such as Li-Fraumeni and Beckwith-Wiedemann syndrome make a person more likely to develop ACT, 4) family history of cancer in relatives of children with ACT, 5) how children with ACT in different parts of the world are treated, and 6) how the genetic makeup affects survival in children and relatives with ACT.

Age:

21 years of age or younger at diagnosis

Adrenocortical tumor care at St. Jude

St. Jude provides the highest quality of care for patients with ACT:

  • Researchers from St. Jude and centers 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 tumor samples to be stored and shared. Researchers use the data to better understand ACT, its risks, and outcomes. It helps doctors all around the world determine the best ways to treat these tumors. Learn how your family can take part.
  • 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. They discuss research and projects, and they discuss 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 patients. These studies may show 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.
  • Jude offers a dedicated team of specialists to meet the needs of children with cancer, including:

More reasons to choose St. Jude for care include:

  • We are consistently ranked among the best childhood cancer centers in the nation by US News & World Report.
  • At St. Jude, we have created an environment where children can be children and families can be together.
  • We lead more clinical trials for childhood cancer than any other hospital in the U.S. 
  • St. Jude is the only National Cancer Institute–designated Comprehensive Cancer Center just for children. A Comprehensive Cancer Center meets rigorous standards for research that develops new and better approaches to prevent, diagnose, and treat cancer.
  • The nurse-to-patient ratio at St. Jude is about 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
  • Patients may be able to get expert, compassionate care and treatment closer to their homes through the St. Jude Affiliate Program.

Contact us

The St. Jude Collaborative Rare Endocrine Clinic Tumor Referral Clinic is now accepting referrals for children with thyroid and adrenocortical tumors. Call 901- 595-4055 or email endocrinetumor.consult@stjude.org for more information.

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Seeking treatment at St. Jude

Patients accepted to St. Jude must have a disease we treat and must be referred by a physician or other qualified medical professional. We accept most patients based on their ability to enroll in an open clinical trial.

How to seek treatment

Contact the Physician / Patient Referral Office

Submit online referral form

Call: 1-888-226-4343 (toll-free) or 901-595-4055 (local)  | Fax: 901-595-4011 | Email: referralinfo@stjude.org | 24-hour pager: 1-800-349-4334

 

Learn more