NCI Definition: A rare, usually large (greater than 5cm), malignant epithelial tumor arising from the adrenal cortical cells. Symptoms are usually related to the excessive production of hormones, and include Cushing's syndrome and virilism in women. Common sites of metastasis include liver, lung, bone, and retroperitoneal lymph nodes. Advanced radiologic procedures have enabled the detection of small tumors, resulting in the improvement of the 5-year survival. [1]

Adrenal cortex carcinomas most frequently harbor alterations in TP53, CTNNB1, CDKN2A, ATRX, and RB1 [2].

Most Commonly Altered Genes in Adrenal Cortex Carcinoma

TP53 Mutation, TP53 c.217-c.1178 Missense, TP53 Missense, CTNNB1 Mutation, and CDKN2A Loss are the most common alterations in adrenal cortex carcinoma [2].

Top Alterations in Adrenal Cortex Carcinoma

Significant Genes in Adrenal Cortex Carcinoma




Disease Details

Adenocarcinoma, Adrenocortical, Malignant, Adrenal Cortex Cancer, Adrenal Cortical Adenocarcinoma, Carcinoma of the Adrenal Cortex, Adrenal Cortex Adenocarcinoma, Adrenal Cortical Carcinoma, Adrenocortical Carcinoma, CARCINOMA, ADRENOCORTICAL, MALIGNANT, cancer of the adrenal cortex, adrenocortical cancer, Adrenocortical carcinoma, NOS, Cortical Cell Carcinoma, Carcinoma of Adrenal Cortex
Malignant Adrenal Cortex Neoplasm
OncoTree Name
Adrenocortical Carcinoma
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.