Adrenal cancer, also called adrenocortical carcinoma (ACC), is a rare cancer that develops in the outer layer (cortex) of the adrenal glands. The adrenal glands are small glands located above each kidney that produce hormones such as cortisol, aldosterone, and adrenal androgens. Adrenocortical cancer may be functioning, meaning it produces excessive amounts of hormones, or non-functioning, meaning it does not produce significant amounts of hormones. Some adrenal cancers are discovered incidentally during imaging tests performed for another medical condition. Adrenal cancer is different from metastatic cancer to the adrenal gland, in which cancer that originated elsewhere in the body spreads to the adrenal gland.
The exact cause of adrenocortical cancer is not known. Research is ongoing to understand how genetic and cellular changes cause normal adrenal cells to become cancerous. Cancer generally develops when changes occur in the DNA of cells, causing them to grow and divide uncontrollably. In some people, these genetic changes may be associated with inherited conditions.
Most people who develop adrenal cancer do not have a clearly identifiable risk factor. However, certain inherited genetic conditions may increase the risk. These include:
Li-Fraumeni syndrome
Beckwith-Wiedemann syndrome
Familial adenomatous polyposis (FAP)
Multiple endocrine neoplasia type 1 (MEN1)
A family history of certain cancers or inherited cancer syndromes may also be relevant. Having a risk factor does not mean that a person will definitely develop adrenal cancer.
The adrenal glands contain different types of tissue, and tumors can develop from different cells. Adrenocortical carcinoma (ACC) develops from the cortex, the outer part of the adrenal gland, and is the primary type of adrenal cancer. Other adrenal tumors include:
Adrenal adenomas: Usually benign (non-cancerous) tumors of the adrenal cortex.
Pheochromocytomas: Tumors arising from hormone-producing cells in the inner part of the adrenal gland (medulla). Most are benign, although some can be malignant.
Neuroblastoma: A cancer that mainly affects children and develops from immature nerve cells, sometimes in or near the adrenal glands.
These conditions are different from adrenocortical carcinoma and may require different treatments.
The doctor will ask about symptoms, hormone-related changes, personal and family medical history, and examine the abdomen and other areas as appropriate.
Blood and urine tests can determine whether the adrenal gland is producing excessive amounts of hormones. Depending on the suspected tumor, testing may include hormone levels such as:
A biopsy is not always necessary before surgery for a suspected adrenocortical carcinoma. In some cases, doctors may avoid biopsy because it may not reliably distinguish certain adrenal tumors and, depending on the situation, could carry a risk of spreading tumor cells along the biopsy tract. The diagnostic approach should therefore be determined by a specialist experienced in adrenal tumors.
Surgical removal of the adrenal gland (adrenalectomy) is the main potentially curative treatment for localized adrenocortical carcinoma. In some cases, surrounding tissues or nearby lymph nodes may also need to be removed if the cancer has invaded them. Because adrenal cancer is rare and complex, surgery is generally best performed by a surgical team experienced in treating adrenal malignancies.
Mitotane is a medication that specifically affects adrenal cortical cells. It may be used after surgery in people at high risk of recurrence and may also be used for advanced disease. Mitotane can affect normal adrenal function, so patients may require careful hormone monitoring and replacement therapy.
Chemotherapy may be recommended for advanced or recurrent adrenal cancer. Combination chemotherapy may be used in certain situations, sometimes together with mitotane.
Radiation therapy may be considered in selected patients, including some people with residual disease, recurrent cancer, or cancer that has spread to specific areas.
If the tumor produces excessive hormones, additional medicines may be required to control the effects of hormone overproduction. For example, treatment may be needed to control:
There is currently no established way to prevent most cases of adrenocortical carcinoma because its exact cause is unknown. People with certain inherited cancer syndromes may benefit from genetic counseling and appropriate medical surveillance. Individuals with a strong family history of related cancers should discuss their risk with a healthcare professional.
The outlook for adrenal cancer varies considerably from person to person. Important factors include:
Stage of the cancer at diagnosis
Tumor size
Whether the cancer has spread
Whether the tumor can be completely removed
Whether the tumor produces hormones
Features of the tumor seen under a microscope
Overall health and response to treatment
When adrenocortical carcinoma is detected early and can be completely removed, treatment may be potentially curative. Advanced or metastatic disease is more difficult to treat and may require a combination of therapies.
No. Many adrenal tumors are benign, including adrenal adenomas. Other tumors, such as pheochromocytomas, are different from adrenocortical carcinoma and can have different biological behavior.
Yes. Some adrenal cancers produce hormones that can raise blood pressure. Cortisol-producing tumors and certain other hormone-producing adrenal tumors may cause hypertension.
Some cases can be cured, particularly when the cancer is localized and can be completely removed with surgery. The likelihood of cure depends strongly on the stage and other characteristics of the cancer.
This article is intended for general educational and informational purposes only. It should not be used to diagnose or treat adrenal cancer or to replace advice from a qualified healthcare professional. Anyone with concerning symptoms, an adrenal mass, or abnormal hormone-test results should seek appropriate medical evaluation.
