Learn About Adrenocortical Carcinoma

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.

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What are the alternative names for Adrenocortical Carcinoma?

Tumor - adrenal; ACC - adrenal

What are the causes of 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 of 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
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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.

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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 of Adrenocortical Carcinoma?

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

When should I contact a medical professional for Adrenocortical Carcinoma?

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

Endocrine glands
Adrenal metastases - CT scan
Adrenal Tumor - CT
What are the latest Adrenocortical Carcinoma Clinical Trials?
Investigation of the Tumorigenesis of Adrenal Tumors and Evaluation of New Therapeutic Options - Untersuchungen Zur Tumorgenese Von Nebennierentumoren Und Evaluation Neuer Therapieoptionen

Summary: The pathogenesis of adrenal tumors is still not fully elucidated and the treatment options for malignant tumors are poor. The current study investigates different aspects of the pathogenesis of adrenal tumors and evaluates different therapeutic options in patients with adrenocortical carcinoma.

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A Phase 1/2 Trial of a Novel Therapeutic Vaccine (EO2401) in Combination With Immune Check Point Blockade, for Treatment of Patients With Locally Advanced or Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma

Summary: This is a multicenter, Phase 1/2, First-In-Human study to assess the safety, tolerability, immunogenicity, and preliminary efficacy of EO2401 in Metastatic Adrenocortical Carcinoma, or Malignant Pheochromocytoma/Paraganglioma.

What are the Latest Advances for Adrenocortical Carcinoma?
Open Versus Laparoscopic Surgery in the Management of Adrenocortical Carcinoma: A Systematic Review and Meta-analysis.
Minimally Invasive Surgery for Resectable Adrenocortical Carcinoma: A Nationwide Analysis.
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A Case Report of a Patient with Metastatic Adrenocortical Carcinoma who Received the Combination Etoposide, Doxorubicin, Cisplatin, and Mitotane Therapy and Achieved Remission.
Who are the sources who wrote this article ?

Published Date: August 15, 2022
Published By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

What are the references for this article ?

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 August 25, 2022. Accessed October 14, 2022.

Yeh MW, Livhits M, Duh Q-Y. The adrenal glands. In: Townsend Jr CM, Beauchamp RD, Evers DM, Mattox KL, et al, eds. Sabiston Textbook of Surgery. 21st ed. St Louis, MO: Elsevier; 2022:chap 40.