Adrenal Cancer Overview

Adrenal cancer is an uncommon type of neuroendocrine tumor (NET) that originates in the adrenal glands. Situated atop the kidneys, these two small, triangular-shaped endocrine glands produce certain hormones, such as adrenaline, cortisol and aldosterone. Once secreted into the bloodstream, these hormones help regulate the body’s metabolism, blood pressure, immune system, stress response and other vital functions.

In the United States, an estimated 200 to 600 people are diagnosed with adrenal cancer each year. When detected early, the tumor can often be successfully treated and sometimes cured.

What is an adrenal mass?

An adrenal mass, or tumor, is an abnormal growth that forms on an adrenal gland. The tumor may be noncancerous (benign) or cancerous (malignant). Additionally, it may be functional (hormone-producing) or nonfunctional. The types of adrenal tumors include:

• Adrenal adenomas – The most common type of benign adrenal tumor, adenomas sometimes produce excess steroid hormones, such as cortisol or aldosterone.
• Myelolipomas – Rare, benign tumors composed of fat and bone marrow-like tissue, myelolipomas are usually nonfunctional and found incidentally.
• Adrenocortical carcinoma – A rare but aggressive cancer that originates in the outer layer (cortex) of the adrenal gland, adrenocortical carcinoma often produces excess hormones, such as cortisol or aldosterone.
• Pheochromocytomas – Usually benign, pheochromocytomas develop in the inner part of the adrenal gland (medulla) and produce excess adrenaline.
• Metastatic adrenal tumors – After developing in another part of the body, such as a kidney, breast or lung, cancer cells can enter the bloodstream or lymphatic system and spread (metastasize) to the adrenal glands.

What causes adrenal cancer?

Adrenal cancer occurs when healthy cells in the adrenal glands undergo harmful changes that disrupt the normally orderly cell growth cycle, leading to rapid cell division and the formation of tumors. The precise mechanisms behind the genetic mutations that cause adrenal cancer are not fully understood.

What are the risk factors for adrenal cancer?

Although rare overall, adrenal cancer has been linked to certain risk factors, including:

• Smoking and other forms of tobacco use
• Long-term exposure to certain pesticides and industrial solvents
• Certain inherited genetic conditions, including Li-Fraumeni syndrome, Lynch syndrome and multiple endocrine neoplasia (MEN1)

While adrenal cancer can occur at any age, it is most often diagnosed before age 5 or between ages 40 and 60.

What are the signs and symptoms of adrenal cancer?

Adrenal cancer may cause noticeable symptoms if a functional tumor is producing excess hormones, which can lead to:

• Changes in sexual desire (libido)
• Frequent mood swings
• High blood pressure (hypertension)
• High blood sugar
• Facial rounding and weight gain in the midsection (Cushing syndrome)
• Muscle weakness and cramping
• Early-onset puberty
• Irregular menstrual cycles in women
• Clitoral enlargement in women
• Penile enlargement in men

Additionally, in women, excessive androgen production may cause vocal deepening and excessive body hair growth. In men, excessive estrogen production may cause breast enlargement or tenderness.

How is adrenal cancer diagnosed?

The diagnostic process for adrenal cancer may include a physical examination and:

• Imaging tests – Computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) scans can help the physician visualize the adrenal glands and detect the presence, size and spread of any tumors.
• Lab tests – Hormone levels can be measured through blood work and urinalysis, which can help the physician identify a functional tumor.
• Biopsy – If adrenal cancer is suspected, the physician may recommend removing the affected adrenal gland, which will be microscopically examined by a pathologist who can identify cancerous cells.
• Adrenal venous sampling (AVS) – Performed by an interventional radiologist, AVS is a minimally invasive outpatient procedure that involves mapping the adrenal veins with contrast medium and drawing blood samples to determine if excess aldosterone is coming from one or both adrenal glands.

How is adrenal cancer staged?

Adrenal cancer is staged using the American Joint Committee on Cancer (AJCC) TNM staging system, which takes into account:

• The size and extent of the primary tumor (T)
• Whether the cancer has reached nearby lymph nodes (N)
• Whether the cancer has metastasized to distant tissues or organs (M)

Adrenal gland cancer stages range from 1 to 4, with higher numbers indicating more advanced tumors.

How is adrenal cancer treated?

Adrenal cancer treatment can vary based on the type, stage and functional status of the tumor and the patient’s overall health. Common options include:

• Surgery – Removal of the affected adrenal gland (adrenalectomy) is the primary treatment for adrenal cancer, which may be curative if the tumor is localized.
• Radiation therapy – High-energy beams are precisely targeted at the tumor to destroy the cancerous cells, which can shrink the tumor and make it easier to remove or control the cancer if the tumor is inoperable.
• Chemotherapy – A systemic treatment, chemo for advanced or metastatic adrenal cancer involves the use of powerful cancer-fighting drugs, such as mitotane, to destroy widespread cancer cells or inhibit their growth.
• Hormone therapy – If the tumor is functional, medications may be used to block or reduce its production of excess hormones, helping to manage any hormone-related symptoms and complications.
• Targeted therapy – Currently under evaluation in clinical trials, novel drugs specifically target the molecular changes or pathways known to promote tumor growth.

Frequently asked questions (FAQs) about adrenal cancer

The following FAQs-related articles provide additional information about adrenal cancer:

• What causes adrenal nodules?
• What is adrenal carcinoma?
• What should I expect with surgery for adrenal carcinoma?

Benefit from world-class care at Moffitt Cancer Center

Because adrenal cancer is exceedingly rare and often challenging to treat, it is essential for a patient to seek specialized care at a high-volume cancer center. The multispecialty team in Moffitt’s nationally recognized Endocrine Oncology Program has the essential knowledge and experience to accurately diagnose and effectively treat adrenal cancer. Our patients have access to multiple experts, advanced technologies and the latest treatment options, including a robust portfolio of clinical trials. We take a comprehensive, individualized approach to adrenal cancer treatment, helping each patient achieve the best possible outcome and quality of life.

If you would like to learn more about adrenal cancer, you can request an appointment with a specialist at Moffitt by calling 1-888-663-3488 or submitting a new patient registration form online. We do not require referrals.

References

National Cancer Institute: Adrenal Gland
American Cancer Society: Key Statistics for Adrenal Cancer
American Cancer Society: Adrenal Cancer Risk Factors
American Cancer Society: Signs and Symptoms of Adrenal Cancers
American Cancer Society: Adrenal Cancer Stages

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