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What are adrenocortical carcinomas?

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 children to develop masculine traits and can spread to the kidneys, lungs, bones and brain. Many children often have Li-Fraumeni syndrome, an inherited condition that predisposes them to many types of cancer.

How we care for adrenocortical carcinomas

Children and adolescents with adrenocortical carcinoma are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Solid Tumor Center. 

Adrenocortical Carcinoma | Symptoms & Causes

What are the symptoms of adrenocortical carcinoma?

The most common symptoms of adrenocortical carcinoma include a palpable abdominal mass and adrenocortical hormone overproduction. In those born male, this hormonal overproduction may cause:

  • premature puberty with enlargement of the penis and scrotum
  • premature growth and increase in muscle mass
  • pubic hair
  • acne
  • deepening voice

In those born female, 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.

What causes adrenocortical carcinoma?

It is important to understand that tumors often emerge with no known cause. Many may result from the combined effects of genetic and environmental factors.

Some cancers are caused by inherited conditions. Disorders associated with pheochromocytomas include neurofibromatosis, von Hippel-Lindau disease, multiple endocrine neoplasia (MEN) syndromes, tuberous sclerosis, Sturge-Weber syndrome, and ataxia-telangiectasia.

Adrenocortical carcinomas are often linked to genetic conditions, most often Li-Fraumeni syndrome. About 50-to-80 percent of pediatric ACC patients have this syndrome. Other related conditions include MEN1, Lynch syndrome, Beckwith-Wiedemann syndrome, and hemihypertrophy.

Adrenocortical Carcinoma | Diagnosis & Treatments

How are adrenocortical carcinoma diagnosed?

In addition to a complete medical history and physical examination, adrenocortical carcinoma is diagnosed with:

After all tests are completed, doctors will be able to outline the best treatment options.

What are the treatment options for adrenocortical carcinoma?

Adrenocortical carcinoma is treated with surgery, chemotherapy or radiation therapy. 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.

What is the long-term outlook for children with adrenocortical carcinoma?

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 percent. Complete surgical removal can cure adrenocortical carcinoma.

Adrenocortical Carcinoma | Programs & Services