Definition and Overview
The adrenal glands are endocrine glands found above each kidney. They produce hormones crucial to keep the body healthy. These include cortisol (a steroid hormone that regulates metabolism and immune response), adrenaline (a hormone responsible for the body’s fight-or-flight response), and aldosterone (stimulates sodium absorption and plays a crucial role in balancing water and salt in the body), among others. The outer part of the adrenal gland is called adrenal cortex while the inner portion is the medulla.
Adrenal carcinoma is a type of cancer that originates from the cortex. Also referred to as adrenocortical or adrenal cortical carcinoma, adrenal cancer is a rare but aggressive type of cancer. Although it accounts for just 0.02% of all cancer-related deaths, the five-year survival rate for advanced cases is only 7%. It commonly affects children under five years old (neuroendocrine tumors) and adults between 30 and 40 years old (neuroblastoma). Due to its aggressiveness and lack of symptoms during its early stages, most cases are diagnosed when the disease has already metastasized to distant organs resulting in poor prognosis. The most common sites of spread are the liver, lungs, bones, lymph nodes, and periadrenal tissues.
A tumor of the adrenal cortex can be non-functioning, which means that they do not produce hormones. However, most adrenal cortex tumors are functioning and produce dangerous levels of certain hormones, causing various symptoms and changes in the body. For example, too much cortisol can cause weight gain, lumps of fat on the back of the neck, and swelling of the sex organs. Extremely high levels of certain hormones also increase the risk of cardiac arrhythmias and high blood pressure.
Causes of Condition
Just like with other types of cancer, the causes of adrenal cancer are still unknown. However, scientists have identified factors that can increase one’s risk of developing the condition. These include cigarette smoking and certain genetic conditions including Li-Fraumeni and Beckwith-Wiedemann syndromes as well as familial adenomatous polyposis and multiple endocrine neoplasia.
The adrenal glands produce regulatory hormones. When cancerous tumors develop, these glands secrete either too little or too much hormones leading to hormonal imbalances. The tumors, which can grow in size, can also put pressure on nearby organs, such as the stomach. This leads to certain symptoms including unexplained weight loss and feeling of fullness. However, in the majority of cases, symptoms are not apparent until the disease has progressed to advanced stages.
Depending on the specific hormone/s affected by the condition, patients may experience any or a combination of the following symptoms:
Deepening of the voice
Growth of breast tissues in men
Growth of fine hair on the arms, upper back, and face
High blood pressure/blood sugar
Lower sex drive
Lump or pain in the abdomen
Pain in the back
Vaginal bleeding during menopause
The early warning signs of adrenal cancer seem unrelated or not very obvious. This makes it difficult to diagnose the condition during its early stages when it is most responsive to treatment.
Who to See and Types of Treatments Available
Patients suffering from symptoms mentioned above are encouraged to consult with a general or family physician for an initial evaluation. If an adrenal tumor or adrenal adenoma is suspected, patients will be referred to an endocrinologist. Endocrinologists are highly trained physicians specializing in the prevention, assessment, diagnosis, and treatment of diseases related to various glands.
Typically, patients undergo thorough assessment during the initial meeting wherein the endocrinologist performs a physical examination and reviews the patient’s medical history. Based on the clinical assessment and the patient’s signs and symptoms, further laboratory tests and radiology studies to examine the blood and urine may be subsequently performed. These may include:
24-hour urine and low-dose dexamethasone suppression tests to measure the amounts of cortisol in the urine. A higher level of cortisol in the urine could be a sign of disease in the adrenal cortex.
Blood chemistry study to measure the amount of potassium or sodium in the body
CT, PET, and MIBG scans
Magnetic resonance imaging (MRI)
Once a malignant adrenal gland tumor has been confirmed, the next step is cancer staging. Determining the stage of cancer is crucial in identifying the best treatment method to be able to provide the most appropriate adrenal support. In this process, various tests are performed to determine the actual size of the tumor and whether or not it has grown into nearby areas or has spread to lymph nodes and distant organs.
As for the treatment, stages I and II adrenal carcinoma are managed with surgery in which the entire adrenal gland is removed. Nearby lymph nodes may also be removed if they are enlarged. In many cases, surgery adequately addresses the condition. However, if cancer cells are not removed completely or if there is a chance that the cancer would come back in the future, patients may also undergo adjuvant therapy such as radiation therapy.
Patients with stage III adrenal carcinoma typically undergo surgery wherein the adrenal glands including nearby lymph nodes and a part of the kidney and liver are removed. They also undergo adjuvant therapy. In the case of stage IV adrenal cancer, surgeons may also elect to surgically remove the tumor with the goal to reduce symptoms rather than to treat the disease. This is typically followed by chemotherapy and radiation therapy.
Other treatment options include ablation in which tumor cells are destroyed if it is unsafe to remove them surgically. The most common drug used in the treatment of adrenal cancer is Mitotane. The drug blocks excessive hormone production and is commonly given after surgery. Patients may also undergo clinical trial treatments, such as biologic therapy, should they qualify.
The five-year survival rate is 65% for those with localized cancer. This goes down to 44% for regional cancer and only 7% when cancer has already metastasized to distant organs.
Adrenal cancer. (n.d.). American Cancer Society. http://www.cancer.org/cancer/adrenalcorticalcancer/detailedguide/index
Adrenocortical carcinoma treatment. (2015, June 26). American Cancer Society. http://www.cancer.gov/cancertopics/pdq/treatment/adrenocortical/Patient/page1
Baker, R. (2015, January 26). Adrenal Tumors: Anatomy, Physiology, Diagnosis, and Treatment. http://www.interventionaloncology360.com/content/adrenal-tumors-anatomy-physiology-diagnosis-and-treatment