Adrenocortical CarcinomaRSS

Last Updated: November 15, 2013

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What is adrenocortical carcinoma?

Adrenocortical carcinoma is a rare cancer that develops in the outer layer of tissue of the adrenal glands, organs that lie on top of each kidney. The outer layer of the adrenal gland, called the adrenal cortex, produces important hormones called steroids that help the body deal with stress, regulate blood pressure and the amount of salt in the blood, as well as cause the body to acquire masculine or feminine characteristics.1 A tumor of the adrenal cortex can produce either no hormones or excess hormones.

In the U.S., an estimated 300 people are diagnosed with adrenocortical carcinoma each year.1 If detected at an early stage, this type of cancer can often be successfully treated. However, almost 70 percent of people are diagnosed with advanced adrenocortical carcinoma.2 For patients at the latest stage of this cancer, less than 20 percent survive five years after diagnosis.

View additional information on adrenocortical carcinoma.

Adrenocortical carcinoma is part of an effort to characterize rare tumor types. Read more about the Rare Tumor Projects.

What types of discoveries about adrenocortical carcinoma do The Cancer Genome Atlas (TCGA) researchers hope to make?

TCGA researchers have:

  • Identified whole genome doubling, wherein a cell has one or more extra sets of chromosomes, as a probable driver of adrenocortical carcinoma
      • Whole genome doubling occurred in 51 percent of adrenocortical carcinoma tumors studied, more than any of twelve other cancer types used for comparison
      • Hypoploidy, or the loss of a significant amount of the genome, was also observed in a higher proportion of cases, 31 percent, than in twelve other cancer types used for comparison with the exception of kidney chromophobe carcinoma
  • Supported previous findings that overexpression of IGF2, a gene encoding a hormone involved in cell growth, and mutation of TP53, a tumor suppressor gene, and the most commonly mutated gene in cancer, are hallmarks of adrenocortical carcinoma
  • Found novel gene mutations that likely contribute to the development of adrenocortical carcinoma
      • Mutations in PRKAR1A found in 8 percent of patients in the study, have previously been associated with tumors and other diseases of the adrenal cortex

See more about TCGA's study of adrenocortical carcinoma

Where can I find more information about the TCGA Research Network's studies, or studies using TCGA data?

View a list of TCGA scientific publications.

Where can I find clinical trials to treat adrenocortical carcinoma that are supported by the National Cancer Institute (NCI)?

View a list of NCI-supported adrenocortical carcinoma clinical trials that are now accepting patients.

Selected References 

1  American Cancer Society. (2012). Adrenal Cancer. Retrieved from

2 Phan AT. (2007) Adrenal cortical carcinoma—review of current knowledge and treatment practices. Hematol Oncol Clin N Am. 21(3):489–507.