What Is Adrenal Carcinoma?

What Is Adrenal Carcinoma?
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Adrenal carcinoma is a rare form of cancer that occurs when healthy cells in the adrenal glands mutate and grow out of control.

 The adrenal glands are two small organs located above each of the kidneys in the upper part of the abdomen. They are responsible for producing various hormones, such as adrenaline and cortisol, that help regulate body functions.

Adrenal carcinomas are often called adrenocortical carcinomas, a reference to where the tumors develop: in the outer part of the adrenal gland called the cortex.

Individual survival rates vary greatly and depend on many factors, including the size and extent of the cancer and your overall health.


Most cancers of the adrenal glands spread from other locations, when other forms of cancer have metastasized and spread.

 This guide is specifically about cancers that originate in the adrenal gland, which is less common.

Types of Adrenal Carcinoma

Adrenal carcinomas are considered “functioning” or “non-functioning,” depending on whether or not they produce excess hormones. An estimated 70 percent of adrenal carcinomas are functioning, producing hormones such as cortisol, androgens (like testosterone), estrogen, or aldosterone.

Though very rare, other types of adrenal tumors include:

  • Neuroblastoma, which starts in neuroblasts (early nerve cells) in the adrenal gland and usually affects infants or children under 10.

  • Pheochromocytoma, which begins in the medulla, the inner part of the adrenal gland, usually in adrenaline-producing cells.

  • Paraganglioma, which develops near certain blood vessels and nerves outside the adrenal glands.

Most adrenal gland tumors or lesions are benign or noncancerous and do not spread beyond the organ. Benign tumors are called adrenal adenomas.

Signs and Symptoms of Adrenal Carcinoma

Adrenal carcinomas can cause a range of symptoms — either from the hormones they overproduce or the tumor itself pressing on other nearby structures. These include:

  • High blood pressure
  • Weight gain, particularly in the cheek area (moon face), around the midsection, and above the collarbone
  • Fatigue
  • Depression and anxiety
  • Insomnia
  • Tremor
  • Lightheadedness when standing up from sitting or lying down
  • Headache
  • Rapid or irregular heartbeat
  • Sweating
  • Paleness
  • Elevated blood sugar levels, which can lead to diabetes
  • Acne

  • Excessive hair growth on the face and body
  • Weakness and loss of muscle, especially in the legs
  • Weakened bones (osteoporosis)
  • Bruising easily
  • Purple-red stretch marks on the abdomen, thighs, or breasts
  • Low blood potassium levels
  • Muscle cramps
  • Abdominal pain or fullness
  • Back pain or discomfort

  • Enlarged penis or breasts in boys
  • Enlarged clitoris or early puberty in girls
  • Deepening voice or menstruation abnormalities in women
  • Breast tenderness or enlargement, lowered sex drive, or erectile dysfunction in men

Causes and Risk Factors of Adrenal Carcinoma

While the exact cause of adrenal cancer remains unclear, several factors may contribute to its development.

Adrenal carcinoma runs in families 50 percent of the time,

 and certain inherited conditions can increase your risk of the disease, including:

  • Li-Fraumeni syndrome
  • Beckwith-Wiedemann syndrome
  • Carney complex

  • Multiple endocrine neoplasia type 1 (MEN1)
  • Multiple endocrine neoplasia type 2 (MEN2)
  • Familial adenomatous polyposis
  • Lynch syndrome
  • Von Hippel-Lindau disease
  • Von Recklinghausen’s disease (neurofibromatosis type 1)
  • Paraganglioma syndrome
Other risk factors include:

  • Overweight or obesity

  • A sedentary lifestyle

  • Smoking
  • Exposure to chemicals like asbestos and radon
  • Radiation therapy to your abdomen

How Is Adrenal Carcinoma Diagnosed?

To diagnose adrenal carcinoma, your doctor will likely perform the following tests:

  • Physical examination, including reviewing your medical history, evaluating your symptoms, reviewing your risk factors, and examining your abdomen for any masses
  • Blood and urine tests to measure the amount of adrenal hormones in your body
  • Dexamethasone suppression tests, which determine if your adrenal gland is producing too much of the hormone cortisol
  • Adrenal angiography and venography, which determine if the blood flow in your adrenal arteries and veins are blocked, respectively
  • Imaging tests to visualize the size and spread of the tumor, such as ultrasound, CT, MRI, PET, and X-ray scans
  • MIGB (meta-iodobenzylguanidine) scans, which use a radioactive tracer to identify pheochromocytomas (a type of tumor) in the inner layer of the adrenal gland

If you have cancer that has spread to other parts of your body (metastasized), your healthcare provider may perform a biopsy — they’ll collect a tissue sample from the tumor and examine it under a microscope — to determine if it is from an adrenal cancer or another type of cancer.

Treatment and Medication Options for Adrenal Carcinoma

Treatment for adrenal carcinoma depends largely on how large it is, how far it has spread, and your general health and fitness.

Surgery

Surgery to remove the tumor is the primary treatment for adrenal carcinoma. During this procedure, called an adrenalectomy, your surgeon may also remove nearby lymph nodes if they are affected.

 In some cases, only a portion of an adrenal gland may need to be removed, while others may require both glands to be fully removed, depending on the location of the cancer.

Your surgeon may use one of two methods to perform an adrenalectomy:



  • Open adrenalectomy is an operation through one large incision or cut in the abdomen.
  • Laparoscopic adrenalectomy is an operation through several smaller incisions using a laparoscope (a long, thin tube with a light and camera at the end).
People who undergo laparoscopic surgery typically report less post-operative pain and faster recovery times, though the procedure requires more skill to complete successfully.

 Some research suggests laparoscopic adrenalectomy carries a higher risk of peritoneal carcinomatosis (cancer of the thin membrane lining the abdominal cavity) than open surgery.

Adrenal cancer returns or recurs after surgery in 19 to 34 percent of cases, so additional treatments (called adjuvant therapies) often follow an adrenalectomy — usually radiation therapy or chemotherapy.

If only one of your adrenal glands is removed, your remaining gland will produce the hormones your body needs. If you have both removed, however, you will require lifetime hormone replacement therapy.

Radiation Therapy

Radiation therapy kills cancer cells using high-energy rays like X-rays.

 Aside from being an adjuvant therapy to adrenalectomy, radiation therapy is also used to reduce symptoms in cases of advanced adrenal carcinoma.

There are a couple types of radiation therapy that may be used to treat adrenal cancer.

  • External-beam radiation therapy, which uses radiation produced from a machine outside the body to kill the cancer cells, usually with imagery and computer guidance systems to target tumor cells accurately with precise dosages.
  • Radiofrequency ablation, which uses a thin, needle-like probe to deliver high-energy radio waves to destroy tumor cells with heat. It’s typically only used if you’re not able to have surgery or the tumor cannot be surgically removed.
Common side effects of radiation therapy include gastrointestinal issues, skin changes like redness and blistering, and fatigue.

Chemotherapy

Chemotherapy, the use of powerful drugs to kill cancer cells, does not work well as a primary treatment for adrenal cancer, but it is useful as an adjuvant therapy or to help manage symptoms of advanced adrenal carcinoma.

The chemo drug usually used for adrenal carcinoma is mitotane, which can both destroy cancer cells in the adrenal gland and block adrenal hormone production.

But mitotane also destroys healthy cells and blocks hormone production in a healthy adrenal gland.

This may result in various symptoms, such as weakness, depression, and high cholesterol.

Hormone Therapy

While mitotane reduces adrenal hormone production, in general, some drugs can help manage elevated levels of specific hormones.

  • For too much cortisol, metyrapone, ketoconazole, mifepristone, and etomidate
  • For too much aldosterone, spironolactone, eplerenone, and amiloride
  • For too much testosterone, bicalutamide, finasteride, and spironolactone
  • For too much estrogen, tamoxifen

Immunotherapy

Immunotherapy drugs help the body’s immune system more effectively find and destroy cancer cells. Immune checkpoint inhibitors are drugs that target specific proteins that cancer cells use to evade the immune system.

Sometimes used with or without mitotane to treat advanced adrenal cancer, pembrolizumab (Keytruda) is an immune checkpoint inhibitor that targets the protein PD-1.

It can cause side effects like cough, gastrointestinal issues, poor appetite, nausea, and skin rash.

It can also cause the immune system to attack other parts of the body, leading to potentially life-threatening issues affecting other organs.


    Prevention of Adrenal Carcinoma

    While not all cases of adrenal carcinoma can be prevented, you can lower your risk with the following measures.

    • Avoid tobacco products, alcohol, and cancer-causing chemicals. The Centers for Disease Control and Prevention recommends that you restrict alcohol consumption to no more than two drinks a day for men and one drink a day for women.

       There is no safe amount of alcohol consumption when it comes to affecting cancer risk — even small amounts could increase risk of health complications or cancer.

    • Maintain a healthy body weight for you. A BMI of 18.5 to 29.4 is generally considered healthy for most adults, but can vary based on body composition.

    • Be physically active. The American Heart Association recommends 150 minutes of moderate to intense physical activity per week for adults.

    • Eat a healthy diet. Stick to meals rich in fruits and vegetables and low in processed meats.
    • Get genetic tests. You may want to consider genetic screening if you have a family history of adrenal cancer or other cancers.

    Lifestyle Changes for Adrenal Carcinoma

    Certain lifestyle changes can improve your overall well-being after having adrenal cancer, such as:

    • Regular checkups and follow-up care. This may include blood and urine hormone tests to check your hormone levels.
    • A healthy, nutritious diet
    • Meditation, yogapsychotherapy, and other stress-reduction practices to help regulate your cortisol (stress hormone) levels

    Adrenal Carcinoma Prognosis

    While the overall five-year survival rate for adrenal carcinoma cancer is 50 percent, five-year survival rates can be further broken down by cancer stage or how far it has spread.

    • 73 percent for localized cancer that’s only growing in the adrenal gland
    • 53 percent for regional cancer that’s spread beyond the gland to nearby lymph nodes or tissues
    • 38 percent for distance cancer that’s spread to the liver, lungs, or other distant parts of the body
    Various factors can affect your specific survival rate, such as your overall health, how early the cancer was detected, and lifestyle factors, such as if you smoke.

    Complications of Adrenal Carcinoma

    Adrenal carcinoma may cause complications related to excess hormone production. The most common of these is hypercortisolism, or excessive cortisol levels, which occurs in 50 to 80 percent of functioning adrenocortical carcinomas.

     This can cause symptoms such as:

    • Diabetes mellitus
    • Fatigue
    • High blood pressure
    • Obesity
    • Muscle weakness and atrophy
    • Osteoporosis

    Research and Statistics: How Many People Have Adrenal Carcinoma?

    While adrenal tumors are relatively common, showing up in about 10 percent of people who have an imaging test of the adrenal gland, the vast majority are benign tumors called adenomas.

    The American Cancer Society estimates that there are only about 200 new cases of adrenocortical carcinomas diagnosed each year in the United States.

    Adrenocortical carcinomas tend to affect more women than men, and it is most often found in young children under 5 years old and adults in their forties to fifties.

    Related Conditions

    There are many conditions that affect the adrenal glands, causing them to produce too little or too much of one or more hormones, such as:

    The Takeaway

    • Adrenal carcinoma is a rare, aggressive condition that can wreak havoc on your hormones, causing a wide range of issues.
    • Understanding the disease’s risk factors, recognizing its many symptoms, and maintaining a proactive approach to your health can make a significant difference in treatment outcomes.
    • If caught early enough, the cancer can be surgically removed before it has spread beyond your adrenal glands.

    Common Questions & Answers

    Is adrenal carcinoma curable?

    Yes, particularly if it’s detected early and surgically removed before it spreads.

    Adrenal carcinoma usually grows rapidly and spreads quickly.

    If one adrenal gland is removed, your other gland can produce the hormones your body needs. If both are removed, you will need lifelong hormone replacement treatment.

    Yes, adrenal tumors can cause spikes in various hormones, including cortisol, testosterone, and estrogen.

    Yes, recurrence is not uncommon for adrenal carcinoma, making regular checkups and follow-up care vital.

    EDITORIAL SOURCES
    Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
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    Daniel Landau, MD

    Medical Reviewer

    Daniel Landau, MD, is a distinguished board-certified hematologist-oncologist with a career that has spanned two eminent institutions: the Orlando Health Cancer Institute and the Medical University of South Carolina. With a specialized interest in genitourinary oncology and hematology, he has been at the forefront of managing both benign and malignant conditions.

    Dr. Landau is a pioneering figure in integrating advanced technology into oncology, having served as a director of telemedicine services. Under his leadership, multiple innovative systems have been designed and piloted, all with a singular focus: enhancing the patient experience.

    Beyond his clinical and technological endeavors, Landau is deeply committed to medical education. He has dedicated significant time and expertise to nurturing the skills of medical students, residents, and fellows, ensuring that the flame of knowledge and compassion burns bright in the next generation of oncologists.

    Joseph Bennington-Castro

    Author

    Joseph Bennington-Castro is a science writer based in Hawaii. He has written well over a thousand articles for the general public on a wide range topics, including health, astronomy, archaeology, renewable energy, biomaterials, conservation, history, animal behavior, artificial intelligence, and many others.

    In addition to writing for Everyday Health, Bennington-Castro has also written for publications such as Scientific American, National Geographic online, USA Today, Materials Research Society, Wired UK, Men's Journal, Live Science, Space.com, NBC News Mach, NOAA Fisheries, io9.com, and Discover.