Adrenal Cancer

About Adrenal Cancer

The type of cancer that develops in the cortex of the adrenal gland is called adrenal cortical carcinoma or just adrenal cancer. This rare type of cancer is also known as adrenocortical cancer (or carcinoma).

Adrenal cancer most often is discovered when:

It is found accidentally on an imaging test done to look for something else.
It makes hormones that cause changes such as weight gain and fluid retention, early puberty in children, or excess facial or body hair growth in women.
It starts causing symptoms because it has gotten very large. Large tumors can press on other organs in the abdomen, causing pain or a feeling of fullness. Generally, adrenal cancers are much larger than adrenal adenomas. An adrenal tumor larger than 5 or 6 centimeters (about 2 to 2 1/2 inches) is assumed to be a cancer. In one study, the average size of an adrenal cancer was about 13 cm (5 inches).

Most cancers found in the adrenal gland did not start there and are not adrenal cancers. Instead, they started in other organs or tissues and then spread (metastasize) through the bloodstream to the adrenal glands. For example, lung cancers, melanomas, and breast cancers often spread to the adrenals. When other cancers spread to the adrenals, they are not considered adrenal cancer. They are named and treated based on the place where they started.

Symptoms

In about half of people with adrenal cancer, symptoms are caused by the hormones made by the tumor. In the other half, symptoms occur because the tumor has grown so large that it presses on nearby organs. Symptoms may be caused by an adrenal tumor or by something else. Getting the proper medical tests is the only way to find out and to get the proper treatment, if needed.

Symptoms may be caused by:

Aldosterone-producing adrenal tumors: high blood pressure, weakness, muscle cramps and low blood potassium levels

Androgen- or estrogen-producing hormones: may cause excessive growth of face or body hair, enlarged sex organs or early puberty.

Excess cortisol production: may cause Cushing Syndrome. Some people have all of these symptoms, but many people with high cortisol levels have only a few.

Possible signs and symptoms include:

Depression and/or moodiness
Easy bruising
Excessive hair growth on the face, chest and back in women
Fat deposits behind the neck and shoulders
High blood sugar levels, often leading to diabetes
High blood pressure
Menstrual irregularities
Purple stretch marks on the abdomen
Weakness and loss of muscle mass in the legs
Weakened bones (osteoporosis), which can lead to fractures
Weight gain, usually greatest above the collarbone, in the cheek area (moon face), and around the abdomen

Pressure on nearby organs: may cause pain near the tumor, a feeling of fullness in the abdomen, or trouble eating because of a feeling of filling up easily.

Risk Factors

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 MLH3, MSH6, TGFBR2, PMS1 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.