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The information on this page was reviewed and approved by
Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was updated on April 2, 2021.

About adrenal cancer

Adrenal cancer is a rare disease, affecting about 600 people in the United States annually, according to the American Society of Clinical Oncology. However, adrenal tumors are found in about one in every 10 people who have an imaging test of the adrenal gland. The majority of adrenal gland tumors are noncancerous (benign tumors) and are called adenomas.

Adrenal cancer is diagnosed when abnormal cells develop in or travel to the adrenal glands, the tiny glands above the kidneys. The disease is most often discovered as a tumor in the glands’ outer part, known as the adrenal cortex, which produces hormones, such as cortisol and aldosterone, and helps the body manage stress and regulate blood pressure.

What causes adrenal cancer?

Although genetic syndromes and hereditary traits have been linked to adrenal cancer, most adrenal cancers occur sporadically, and triggers for the disease remain largely unknown.

Some genetic syndromes that are considered risk factors for adrenal cancer include:

  • Li-Fraumeni syndrome
  • Von Hippel-Lindau disease
  • Multiple endocrine neoplasia type 1 and 2 (MEN1, MEN2)
  • Beckwith-Wiedemann syndrome

Conn’s and Cushing’s syndromes may result in adrenal gland tumors, though these tumors tend to be nonfunctioning adenomas.

Learn more about risk factors for adrenal cancer

Who gets adrenal cancer?

Tumors can occur at any age. Adrenal cancer is most common in middle-aged adults, with 46 being the average age of diagnosis. One form of adrenal cancer, neuroblastoma, usually affects infants or children under 10.

Adrenal cancer types

The adrenals are small, triangular glands that sit on top of each kidney and function as part of the endocrine system. The three common types of adrenal cancer affect different parts of the adrenal glands:

  • Adrenocortical carcinoma (also called adrenal cortex cancer, adrenocortical cancer,  adrenal cortical carcinoma or ACC) forms in the outer layer of the adrenal gland
  • Pheochromocytoma forms in the inner part of the adrenal medulla
  • Neuroblastoma forms in nerve cells of adrenal medulla

Learn more about adrenal cancer types

Adrenal cancer symptoms

Most adrenal gland tumors are discovered during scans for unrelated conditions. Sometimes, the only way to distinguish a functioning tumor from a more common benign tumor is to determine whether it has metastasized, with cancer cells spreading beyond the adrenal gland to the lymph nodes or other parts of the body. Tumors larger than 5 to 6 centimeters are usually diagnosed as cancerous.

Symptoms vary by the stage of the tumor, and warning signs are usually not obvious. General symptoms of adrenal cancer include:

  • Fever
  • A noticeable lump in the abdomen
  • Persistent pain (pressure of tumor on organs)
  • Feeling of fullness, caused by a tumor pressing against the stomach
  • Unexplained weight loss or weight gain
  • High blood pressure, heart palpitations, excessive sweating, anxiety
  • Abnormal hormone levels, which can cause excess hair growth or early puberty

In some cases, malignant tumors can produce hormones that lead to an additional set of symptoms called paraneoplastic syndromes. For patients with these syndromes, the immune system responds to the cancerous tumor by attacking healthy cells in the brain, spinal cord, nerves or muscles.

Learn more about symptoms of adrenal cancer

Diagnosing adrenal cancer

Blood and urine tests that look for irregular levels of adrenal hormones may detect adrenal cancer before symptoms develop. Other tests used for diagnosing adrenal cancer—depending on factors such as the tumor’s size and location—include:

Learn more about diagnostic procedures for adrenal cancer

Treating adrenal cancer

According to the American Cancer Society, the 5-year survival rate for adrenal cancer ranges from 31% for cancer that has spread to distant parts of the body to 74% for cancer that has not spread outside beyond the adrenal gland.

Multiple treatment options or treatment combinations may be used for this type of cancer, depending on the stage of the disease. Those treatments options include:

  • Laparoscopy
  • Posterior, transabdominal or thoracoabdominal surgery
  • Adrenalectomy
  • Radiation therapy
  • Chemotherapy

Learn more about treatments for adrenal cancer