What is the definition of Adrenocortical Carcinoma?

Adrenocortical carcinoma (ACC) is a cancer of the adrenal glands. The adrenal glands are two triangle-shaped glands. One gland is located on top of each kidney.

What are the alternative names for Adrenocortical Carcinoma?

Tumor - adrenal; ACC - adrenal

What are the causes for Adrenocortical Carcinoma?

ACC is most common in children younger than 5 years old and adults in their 40s and 50s.

The condition may be linked to a cancer syndrome that is passed down through families (inherited). Both men and women can develop this tumor.

ACC can produce the hormones cortisol, aldosterone, estrogen, or testosterone, as well as other hormones. In women the tumor often releases these hormones, which can lead to male characteristics.

ACC is very rare. The cause is unknown.

What are the symptoms for Adrenocortical Carcinoma?

Symptoms of increased cortisol or other adrenal gland hormones may include:

  • Fatty, rounded hump high on the back just below the neck (buffalo hump)
  • Flushed, rounded face with pudgy cheeks (moon face)
  • Obesity
  • Stunted growth (short stature)
  • Virilization -- the appearance of male characteristics, including increased body hair (especially on the face), pubic hair, acne, deepening of the voice, and enlarged clitoris (females)

Symptoms of increased aldosterone are the same as symptoms of low potassium, and include:

  • Muscle cramps
  • Weakness
  • Pain in the abdomen

What are the current treatments for Adrenocortical Carcinoma?

Primary treatment is surgery to remove the tumor. ACC may not improve with chemotherapy. Medicines may be given to reduce production of cortisol, which causes many of the symptoms.

What is the outlook (prognosis) for Adrenocortical Carcinoma?

The outcome depends on how early the diagnosis is made and whether the tumor has spread (metastasized). Tumors that have spread usually lead to death within 1 to 3 years.

What are the possible complications for Adrenocortical Carcinoma?

The tumor can spread to the liver, bone, lung, or other areas.

When should I contact a medical professional for Adrenocortical Carcinoma?

Call your provider if you or your child has symptoms of ACC, Cushing syndrome, or failure to grow.

Endocrine
Adrenal
Adrenal

REFERENCES

Allolio B, Fassnacht M. Adrenocortical carcinoma. In: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Adult and Pediatric. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 107.

National Cancer Institute website. Adrenocortical carcinoma treatment (Adult) (PDQ) - health professional version. www.cancer.gov/types/adrenocortical/hp/adrenocortical-treatment-pdq. Updated November 13, 2019. Accessed October 14, 2020.

Clinical Trial
  • Status: Not yet recruiting
  • Phase: Phase 1
  • Intervention Type: Drug
  • Participants: 32
  • Start Date: December 2021
B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell Therapy for Pediatric Patients With Solid Tumors (3CAR)
Clinical Trial
  • Status: Recruiting
  • Intervention Type: Other
  • Participants: 180
  • Start Date: January 2, 2021
Vessels Encapsulating Tumor Clusters (VETC), Prognostic and Predictive Value in Renal Cell Carcinoma and Adrenal Gland Carcinoma