Skip to main content

ACS & ASCO are Stronger Together: Cancer.Net content is now available on



Adrenal Cancer Risk Factors

A risk factor is anything that changes your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.

Scientists have found few risk factors that make a person more likely to develop adrenal cancer. Even if a patient does have one or more risk factors for adrenal cancer, it is impossible to know for sure how much that risk factor contributed to causing the cancer.

But having a risk factor, or even several, does not mean that you will get the disease. Many people with risk factors never develop adrenal cancer, while others with this disease may have few or no known risk factors.

Genetic syndromes

The majority of adrenal cortex cancers are not inherited (sporadic), but some (up to 15%) are caused by a genetic defect. This is more common in adrenal cancers in children.

Li-Fraumeni syndrome

The Li-Fraumeni syndrome is a rare condition that is most often caused by a defect in the TP53 gene. People with this syndrome have a high risk of several types of cancer, including include breast cancer, bone cancer, brain cancer, and adrenal cortex cancer.

Beckwith-Wiedemann syndrome

People with this problem have large tongues, are large themselves, and have an increased risk for developing cancers of the kidney, liver and adrenal cortex.

Multiple endocrine neoplasia (MEN1)

People with MEN1 have a very high risk of developing tumors of 3 glands: the pituitary, parathyroid, and pancreas. About one-third to one-half of people with this condition also develop adrenal adenomas (benign tumors) or enlarged adrenal glands. These usually do not cause any symptoms. This syndrome is caused by defects in a gene called MEN1. People who have a family history of MEN1 or pituitary, parathyroid, pancreas, or adrenal cancers should ask their doctor if they might benefit from genetic counseling.

Familial adenomatous polyposis (FAP)

People with this syndrome develop hundreds of polyps in the large intestine. These polyps will lead to colon cancer if the colon is not removed. FAP also increases the risk of other cancers, and may increase the risk for adrenal cancer. Still, most adrenal tumors in patients with FAP are benign adenomas. This syndrome is caused by defects in a gene called APC.

Lynch syndrome or hereditary nonpolyposis colorectal cancer (HNPCC)

Lynch syndrome (formerly known as HNPCC) is an inherited genetic disorder that increases the risk of colorectal cancer, stomach cancer, and some other cancers, including adrenal cortex cancer. In most cases, this disorder is caused by a defect in either the MLH1 or MSH2 gene, but other genes can cause Lynch syndrome, including MLH3MSH6TGFBR2PMS1, and PMS2. Lynch syndrome is discussed in more detail in Colorectal Cancer.

Lifestyle and environmental factors

Risk factors such as being overweight, smoking, living a sedentary lifestyle, and being exposed to cancer-causing substances in the environment can affect a person's risk of many types of cancer. Although none of these factors has been found to definitely influence a person's risk of developing adrenal cancer, smoking has been suggested as a risk factor by some researchers.

The American Cancer Society medical and editorial content team

Our team is made up of doctors and oncology certified nurses with deep knowledge of cancer care as well as editors and translators with extensive experience in medical writing.

Analysis of rare endocrine cancer reveals novel genetic alterations [press release]. National Cancer Institute (NCI) Press Office; May 9, 2016. Accessed at: on December 13, 2017.

Lalli E, Sasano H. 5th International ACC Symposium: An outlook to current and future research on the biology of adrenocortical carcinoma: Diagnostic and therapeutic applications. Horm Can. 2016;7:44-48.

Lirov R, Tobias E, Lerario AM, Hammer GD. Adrenal tumors In: DeVita VT, Lawrence TS, Rosenberg SA, eds. DeVita, Hellman, and Rosenberg’s Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia, PA: Lippincott Williams & Wilkins 2015: Chapter 84.

National Comprehensive Cancer Network. NCCN Clinical Practice Guidelines in Oncology: Neuroendocrine Tumors. v.3.2017. Accessed at
on December 13, 2017.

Online Mendelian Inheritance in Man, OMIM (TM). McKusick-Nathans Institute of Genetic Medicine, Johns Hopkins University (Baltimore, MD) and National Center for Biotechnology Information, National Library of Medicine (Bethesda, MD), 07/26/16. Available at

Raymond VM, Everett JN, Furtado LV, et al. Adrenocortical carcinoma is a Lynch syndrome-associated cancer. J Clin Oncol. 2013;31(24):3012-3018. Epub 2013 Jul 22.

Schneider DF, Mazeh H, Lubner SJ, Jaume JC, Chen H. Cancer of the endocrine system In: Neiderhuber JE, Armitage JO, Doroshow JH, Kastan MB, Tepper JE, eds. Abeloff’s Clinical Oncology. 5th ed. Philadelphia, PA. Elsevier: 2014: 1112-1142.

Last Revised: January 2, 2018

American Cancer Society Emails

Sign up to stay up-to-date with news, valuable information, and ways to get involved with the American Cancer Society.