Adrenal Cancer Risk Factors

A risk factor is anything that increases your chances of getting a disease, such as adrenal cancer. Having a risk factor, or even many, does not mean that you  will get the disease.  Many people with risk factors never develop adrenal cancer, while others with this disease have few or no known risk factors.

Certain adrenal cancer risk factors that are related to personal behaviors, such as smoking can be changed. Others, like a person’s age, genetics, and family history can’t be changed.

Genetic syndromes

Most adrenal cortex cancers are not inherited (they are 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 developing several types of cancer, including breast cancerbone cancerbrain cancer, and adrenocortical cancer.

Beckwith-Wiedemann syndrome

People with Beckwith-Wiedemann syndrome have large tongues, are tall, and have an increased risk for developing cancers of the  kidneyliver  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 tumors. In most cases, these adrenal tumors are adenomas (benign) and non-functional (do not make hormones). However, some adenomas might be functional and make hormones, such as aldosterone and cortisol. Adrenal adenomas 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 patients may also develop tumors elsewhere, like the adrenal glands. But, most adrenal tumors in people 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 adrenocortical 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   MSH6, PMS2, and EPCAM.

Lifestyle and environmental factors

Risk factors such as being overweight, 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. Smoking, in particular, has been suggested as a risk factor for adrenal cancer.

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References

Developed by the American Cancer Society medical and editorial content team with medical review and contribution by the American Society of Clinical Oncology (ASCO).

Analysis of rare endocrine cancer reveals novel  genetic alterations [press release].  National Cancer Institute (NCI) Press Office; May 9, 2016. Accessed at:  https://www.cancer.gov/news-events/press-releases/2016/TCGA-adrenocortical on  July 17, 2024.

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, Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, Version 2.2024--August 1, 2024. Accessed at https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf on July 17, 2024.

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.

Shiroky JS, Lerner-Ellis JP, Govindarajan A, Urbach DR, Devon KM. Characteristics of Adrenal Masses in Familial Adenomatous Polyposis. Dis Colon Rectum. 2018 Jun;61(6):679-685. 

 

 

Last Revised: October 1, 2024

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