Risks for adrenal gland cancer

Some things can affect your risk, or chance, of developing cancer. Certain behaviours, substances or conditions can increase or decrease the risk. Most cancers are the result of many risks. But sometimes cancer develops in people who don't have any risks.

Adrenal gland cancer is rare. Women are slightly more likely than men to develop adrenal gland cancer. We need more research and can't say at this time what the risk is for transgender, non-binary and gender-diverse people.

People who have certain genetic conditions have a higher than average risk for adrenal gland cancer. A genetic condition is a disease caused by a change (mutation) in one or more genes. About 15% of adrenocortical carcinomas (ACC), the most common type of adrenal gland cancer, are linked with a genetic condition. And about 30% of pheochromocytomas, the second most common type of adrenal gland tumour, are linked with a genetic condition.

The following genetic conditions can increase your risk for adrenal gland cancer. If you have a genetic condition that increases your risk for adrenal gland cancer, you may need to visit your doctor more often. Talk to your doctor about your risk and if you need to have certain tests to check for adrenal gland cancer.

Li-Fraumeni syndrome

Multiple endocrine neoplasia (MEN)

Von Hippel-Lindau (VHL) syndrome

Neurofibromatosis type 1

Beckwith-Wiedemann syndrome

Carney complex

Familial adenomatous polyposis (FAP)

Lynch syndrome

Hereditary paraganglioma-pheochromocytoma syndrome

Li-Fraumeni syndrome is an inherited condition that increases the risk of developing certain types of cancers, including ACC, breast cancer, brain tumours, soft tissue and bone sarcomas, and acute leukemia.

People with Li-Fraumeni syndrome tend to develop several different types of cancer before the age of 45.

Multiple endocrine neoplasia (MEN) is an inherited condition that increases the risk of developing endocrine system cancers. It is associated with tumours in more than one endocrine gland.

There are 2 different types of MEN:

• MEN1 increases the risk of ACC and cancer in the parathyroid glands, pituitary gland, pancreas and ovaries.
• MEN2 increases the risk of pheochromocytoma and cancer in the thyroid and parathyroid glands.

Von Hippel-Lindau (VHL) syndrome is an inherited condition that affects blood vessels in different parts of the body, including the eyes, brain, spinal cord and adrenal glands. It makes the blood vessels grow abnormally.

VHL syndrome increases the risk of pheochromocytoma and cancer in the kidneys, brain and pancreas.

Neurofibromatosis type 1 is an inherited condition that affects the nervous system. It affects the development and growth of neurons (nerve cells), causes tumours (neurofibromas) to grow on nerves and may cause other abnormalities in muscles, bones and skin. Sometimes cancerous tumours called malignant peripheral nerve sheath tumours grow along the nerves.

Neurofibromatosis type 1 increases the risk for pheochromocytoma, neuroendocrine tumours, soft tissue sarcoma, brain tumours, leukemia and neuroblastoma.

Beckwith-Wiedemann syndrome is an inherited condition that affects how different parts of the body grow. Signs include large body size, large tongue, large organs, a defect in the abdominal wall and low blood sugar in newborns. Beckwith-Wiedemann syndrome increases the risk of developing cancer, including ACC, Wilms tumour, rhabdomyosarcoma and neuroblastoma.

Carney complex is an inherited condition that affects the skin and causes tumours in the heart, skin, nerves and endocrine glands (such as the thyroid, pituitary gland, testicles and ovaries). It also increases the risk of ACC.

Familial adenomatous polyposis (FAP) is an inherited condition that causes hundreds to thousands of polyps to develop, mainly in the lining of the colon and rectum. People with FAP have a higher risk of developing colorectal cancer and small intestine cancer. FAP also increases the risk of ACC, stomach cancer and thyroid cancer.

Lynch syndrome is an inherited condition that causes a large number of polyps to develop in the lining of the colon and rectum, but not as many polyps as are found in FAP. Lynch syndrome is also called hereditary non-polyposis colorectal cancer (HNPCC). Lynch syndrome increases the risk of ACC and colorectal, uterine and ovarian cancers.

Hereditary paraganglioma-pheochromocytoma syndrome is an inherited condition that causes one or more tumours to grow in paraganglia. Paraganglia are groups of cells that are found near nerve cell bunches called ganglia. A tumour in paraganglia is called a paraganglioma. Hereditary paraganglioma-pheochromocytoma syndrome increases the risk of pheochromocytoma, which is a type of paraganglioma that develops in the adrenal gland.

To make the decisions that are right for you, ask your doctor questions about risks.