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Adrenocortical tumor (ACT) is a rare tumor of the adrenal gland. These tumors can range from benign tumors to aggressive, malignant cancers. Other names for ACT are adrenocortical cancer, cancer of the adrenal cortex, adrenocortical adenoma, and adrenocortical carcinoma.
The adrenal glands are located on top of each kidney. The outer layer of the adrenal gland is called the adrenal cortex. The job of the adrenal glands is to produce hormones such as cortisol and aldosterone. These hormones have a variety of important functions that include:
The adrenal cortex also produces small amounts of male and female sex hormones, called androgens and estrogens. These hormones influence the development of male and female characteristics.
Adrenocortical cancer is very rare in children. It makes up only 0.2% of childhood cancers. In the United States, there are about 25 new cases every year.
In adults, adrenocortical tumors are classified as adrenocortical carcinoma (malignant) or adrenocortical adenoma (benign). However, in children, it can be hard to determine the type of adrenocortical tumor, and aggressiveness can be harder to predict. Therefore, the treatment plan for ACT in children relies on recognizing a potential spectrum of aggressiveness in such tumors, and considering tumor biology as well as clinical course, rather than strictly relying on whether a tumor is initially classified as a “carcinoma” or “adenoma.”
Treatment for adrenocortical tumor usually includes surgery to remove the tumor and adrenal gland. There is a very good chance of cure with complete resection of the tumor by surgery. If the cancer spreads outside the adrenal gland, then treatment will include chemotherapy.
Adrenocortical tumors are more common in girls than boys. This cancer occurs most often in younger children, usually between 1 and 4 years old.
Certain hereditary factors may increase risk, and the tendency to develop ACT may be passed down in families.
Hereditary syndromes that increase risk for adrenocortical tumor include Li-Fraumeni Syndrome (TP53 gene) and Beckwith-Wiedemann Syndrome. Children with these conditions will undergo regular screening including abdominal ultrasound to watch for adrenocortical tumor.
Most children with adrenocortical tumor have signs and symptoms due to excess hormones. If the cancer causes more hormones to be made than normal, it is called functioning ACT. In children, most (90%) of adrenocortical tumors are “functioning.” In teens, about half of adrenocortical tumors are “functioning.”
Other general signs and symptoms of adrenocortical tumor include:
|Hormone-Related Signs and Symptoms of Pediatric Adrenocortical Tumor (ACT)
|Excess androgens or estrogens can produce early signs of puberty or
changes not usually seen in children of that gender
|Male characteristics such as facial and body hair, acne, deepening voice, increased growth
||Female characteristics such as breast development
||Rounded “moon” face, weight gain, fatty hump on upper back, stunted height, high blood sugar, high blood pressure
||High blood pressure, thirst, muscle cramps or weakness
Doctors look for adrenocortical tumors in several ways. These include:
The tumor will be examined and classified according to the histology, mainly as adenoma or carcinoma. However, in children, adrenocortical tumors can have a range of behavior. Not all carcinomas are similarly aggressive, and some tumors initially described as adenomas may have features that are more invasive or need more follow-up than expected.
The stage, or extent of disease, of adrenocortical tumor depends on several factors:
More than half of patients with adrenocortical tumor have metastatic disease at the time of diagnosis. The most common places for the disease to spread are the liver and lungs. ACT may also spread to lymph nodes, bones, and other places in the abdomen.
Recovery from ACT depends on whether surgery completely removes the tumor. Chance of survival is higher with complete resection, or full removal of cancer by surgery. If caught early and with successful surgery, the five-year overall survival for adrenocortical tumor is 85%. For more advanced cancer, survival rate is less than 40%. All patients require long-term monitoring and care by a multidisciplinary team.
Factors that influence chance of cure include:
Surgery to remove the adrenal gland (adrenalectomy) is the main treatment for adrenocortical tumors. When possible, the goal of treatment is complete removal (resection) of the tumor. This type of tumor requires special care during surgery. The adrenal gland is delicate (friable) and can break apart easily during surgery. If parts of the tumor break off (tumor spill), then the chance for spread of the cancer is high. For this reason, a biopsy is not usually used to diagnose ACT before surgery.
Management and pre-operative planning by expert surgeons familiar with pediatric adrenocortical tumors is highly recommended for children with suspected ACT. A multidisciplinary team, including oncologists, endocrinologists and anesthesia specialists, is also important for peri-operative care to help manage blood pressure and changes in electrolytes, body fluids and hormones that may be affected by a large or hormone-producing adrenal mass.
A pathologist will examine the tumor after it is removed with surgery. A multidisciplinary team can then review the findings and conduct additional tests as needed to complete the staging and tailor the care plan for the patient.
If the tumor is completely removed, there is a good chance for cure. For patients with small tumors, surgery alone may be an effective treatment.
Radiation therapy may be considered for adrenocortical tumor, especially if the cancer has spread. Before starting radiation therapy, patients should be tested for the TP53 gene mutation.
Patients with advanced cancer who do not respond to other treatments might consider participating in a clinical trial.
Hormone monitoring and therapy can help patients who have abnormal levels of hormones due to the tumor or treatment. Medicines can be given to help relieve some of the problems of hormone imbalance due to a functioning tumor (extra hormones) or to restore hormone levels that have been affected by the tumor or other treatments.
Patients may need hormone replacement therapy after removal of the adrenal gland and/or treatment with mitotane or other chemotherapy which can affect hormone production. Low adrenal hormones can cause a variety of problems including problems regulating blood pressure and blood sugar.
ACT is a complex disease, and a multidisciplinary care team is important including pediatric oncology, diagnostic imaging, surgery, pathology, radiation oncology, endocrinology, nutrition, and genetic counseling.
All ACT patients ages 21 and younger are invited to participate in an observational study registry of this rare cancer:
Adrenocortical tumor is a very rare cancer in children. Children with this cancer need ongoing care to monitor hormone levels and watch for cancer recurrence.
Endocrine function monitoring
Monitoring of endocrine function in patients with ACT is important. Patients with functioning tumors need medicines to treat symptoms of overproduction. People who have lower adrenal gland function need hormones to help regulate the body’s normal functions, including the response to stress.
Genetic testing and counseling
Children with adrenocortical tumor should be tested for the TP53 gene. People with a germline mutation in this gene have a condition called Li-Fraumeni Syndrome. Family members of patients with an identified mutation should also be tested. The TP53 gene is located in cells throughout the body. Its job is to tell cells to make a tumor suppressor protein that keeps bad or damaged cells from dividing. A change in TP53 puts people at higher risk for certain cancers because the signal to stop “bad” cells does not work correctly. People with a mutation in this gene should avoid exposure to radiation whenever possible. Lifelong monitoring is important because of high cancer risk.
For general health and disease prevention, all cancer survivors should adopt healthy lifestyle and eating habits, as well as continue to have regular physical checkups and screenings by a primary physician. Childhood cancer survivors treated with systemic chemotherapy should be monitored for acute and late effects of therapy.
Survivors treated with systemic chemotherapy or radiation should be monitored for acute and late effects of therapy. Serious chronic health conditions are present 25 years after diagnosis in approximately 25% of survivors, according to the Childhood Cancer Survivor Study. These conditions include second cancers (increased risk following exposure to radiation), congestive heart failure (exposure to doxorubicin), infertility or complications during pregnancy, and end-stage renal disease or renal failure.
Reviewed: June 2018