Adrenal cancer, also known as adrenal cortical carcinoma, is a rare type of cancer that develops in the adrenal glands, which are located on top of the kidneys. The adrenal glands produce hormones that regulate various bodily functions such as blood pressure, metabolism, and stress response.
Types of Adrenal Cancer
There are two main types of adrenal cancer –
- Adrenocortical carcinoma (ACC): This type of cancer arises from the cells in the outer layer (cortex) of the adrenal gland. Adrenocortical carcinoma is rare cancer, and it tends to grow and spread quickly. It can be functional, meaning it produces hormones, or non-functional, meaning it does not produce hormones.
- Pheochromocytoma: This is a rare type of tumor that develops in the chromaffin cells of the adrenal gland. Pheochromocytomas are usually noncancerous (benign), but they can also be cancerous (malignant). They produce excess amounts of catecholamines, which can cause high blood pressure, headache, sweating, and other symptoms.
Adrenocortical carcinoma is the more common type of adrenal cancer, accounting for about 80% of all cases, while pheochromocytoma accounts for only about 5% of cases. In some cases, adrenal cancer may be a metastatic tumor, meaning it has spread to the adrenal gland from another part of the body. In these cases, the cancer is not considered adrenal cancer, but rather a metastasis of another type of cancer.
Causes and Risk Factors
The causes of adrenal cancer are not yet fully understood, but there are several risk factors that may increase the likelihood of developing the disease. These include –
- Genetic mutations: Inherited genetic mutations, such as mutations in the TP53 or RB1 genes, are associated with an increased risk of adrenal cancer.
- Li-Fraumeni syndrome: This is an inherited genetic disorder that increases the risk of developing several types of cancer, including adrenal cancer.
- Beckwith-Wiedemann syndrome: This is another genetic disorder that increases the risk of adrenal cancer, as well as other types of cancer.
- Gender: Adrenal cancer is slightly more common in women than in men.
- Age: The risk of developing adrenal cancer increases with age, and most cases are diagnosed in people over 40.
- Smoking: There is some evidence to suggest that smoking may increase the risk of adrenal cancer.
- Exposure to certain chemicals: Certain chemicals, such as asbestos, may increase the risk of adrenal cancer.
- History of cancer: People who have had cancer in the past may be at a slightly higher risk of developing adrenal cancer.
Having one or more of these risk factors does not necessarily mean that a person will develop adrenal cancer, and many people with adrenal cancer have no known risk factors.
The symptoms of adrenal cancer can vary depending on the size and location of the tumor, as well as whether or not it is producing hormones. Some common symptoms of adrenal cancer may include –
- Abdominal pain or swelling: A mass or tumor in the adrenal gland may cause abdominal pain or a noticeable lump in the abdomen.
- Unexplained weight loss: Adrenal cancer can cause unexplained weight loss, which may be accompanied by loss of appetite and fatigue.
- Hormonal changes: Adrenal tumors can produce hormones that affect the body’s metabolism and hormone balance. For example, some tumors may produce excess amounts of cortisol, which can cause symptoms such as high blood pressure, muscle weakness, and mood changes. Others may produce excess amounts of androgens, which can cause symptoms such as acne, excessive hair growth, and menstrual irregularities.
- Back pain: Adrenal cancer can cause back pain that is often described as a dull ache in the mid-back.
- Nausea and vomiting: Some people with adrenal cancer may experience nausea and vomiting, especially if the tumor is causing gastrointestinal problems.
- High blood pressure: Adrenal tumors can produce hormones that increase blood pressure, leading to hypertension.
These symptoms can be caused by other medical conditions, and having one or more of these symptoms does not necessarily mean that a person has adrenal cancer. If you are experiencing any of these symptoms, it’s necessary to speak with a healthcare provider for an accurate diagnosis and appropriate treatment.
The stages of adrenal cancer are determined by the size of the tumor, whether it has spread to nearby lymph nodes or other organs, and whether it has metastasized to distant parts of the body. The staging system commonly used for adrenal cancer is the TNM staging system, which stands for Tumor, Node, and Metastasis. The stages are as follows –
Stage 1: The tumor is small, measuring 5 centimeters (cm) or less, and has not spread to nearby lymph nodes or other organs.
Stage 2: The tumor is larger than 5 cm but has not spread to nearby lymph nodes or other organs.
Stage 3: The tumor has grown into nearby tissues, such as the kidney or surrounding fat, and may have spread to nearby lymph nodes.
Stage 4: Cancer has spread (metastasized) to distant parts of the body, such as the lungs, liver, bones, or brain.
The diagnosis of adrenal cancer is determined by stage and type of cancer by evaluating medical history and conducting a physical exam of a person. The tests performed for diagnosing cancer will be –
- Imaging tests: Imaging tests such as computed tomography (CT) scan, magnetic resonance imaging (MRI), or positron emission tomography (PET) can be used to detect the presence of a tumor in the adrenal gland, as well as to determine the size and location of the tumor.
- Blood tests: Blood tests may be done to check for abnormal levels of hormones in the blood that may indicate the presence of adrenal cancer. These tests may include the measurement of cortisol, aldosterone, and other hormones produced by the adrenal gland.
- Biopsy: A biopsy involves taking a sample of tissue from the adrenal gland for examination under a microscope. This can help determine if the tumor is cancerous, as well as the type and stage of cancer. Biopsies can be done with a needle biopsy, which is a minimally invasive procedure, or with a surgical biopsy, which involves removing a portion of the adrenal gland.
In some cases, adrenal cancer may be found incidentally during an imaging test for diagnosis of another condition. If adrenal cancer is suspected, it’s important to consult with a healthcare provider who specializes in the treatment of this type of cancer treatments.
Early detection of the condition can help in effective treatment. There are several types of treatments for adrenal cancer including –
- Surgery – A procedure called adrenalectomy may be performed by doctors for the removal of affected adrenal glands. If cancer spreads to other parts of the body then they may also remove nearby lymph nodes and tissues.
- Radiation Therapy – It uses radiation with high-energy rays to kill cancer cells and stop the growth of new cancer cells.
- Chemotherapy – It is administered orally or injected through veins. It is a cancer therapy that stops the growth of cancer cells. It can also be used in combination with other types of treatments depending on the stage of cancer.
- Hormone therapy: Hormone therapy involves the use of drugs to block the production of hormones that may be produced by the tumor. This type of therapy is used in cases where the tumor is producing excess hormones, such as in cases of adrenocortical carcinoma.
Ablation or destruction of tumor cells is performed when the surgery can happen to be unsafe in some patients. Mitotane is a common drug specifically used to kill cancer cells. It is given after surgery to block excessive hormone production that will decrease the size of a tumor.
Participating in a clinical trial may be an option for some patients such as in biologic therapy where the person’s immune system is boosted to fight against cancer cells.
The long-term outlook, or prognosis, for adrenal cancer, depends on several factors, including the stage and type of cancer, the size and location of the tumor, whether or not it has spread to other parts of the body, and the overall health of the patient.
If you have been diagnosed with adrenal cancer, it’s important to work closely with your healthcare provider to determine the best treatment plan for your individual case and also to discuss your long-term outlook.