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What is ACC?

Adrenal cortical carcinoma/cancer or adrenocortical carcinoma, is an aggressive cancer originating in cortex of the adrenal gland. These cancers are extremely rare.  The adrenal glands are two small walnut sized glands shaped like pyramids that sit on top of each kidney, and that make and secrete hormones.  These hormones affect every tissue, gland and organ in the body. 

ACC is a rare tumor, with incidence of approximately 1 in every one million people annually.  In the United States, about 300-500 people are diagnosed every year. They usually occur in only 1 adrenal gland, but can occur in both.  There are two main age groups in which ACC is observed- in children under 5 years of age and in adults between the ages of 40 and 50, and is more common in females (as published in Cancer: principles and practice of Oncology).  No genetic link has been found for the development of ACC.  The overall 5-year survival rate is only 20-35%.
 
Normal cells grow, divide, and die in a controlled way that is regulated in the body. Cancer is a disease that causes cells to divide and grow in an abnormal, out of control way where the balance of cell growth and death is disturbed.  Cells that continue to grow and divide without control become a mass or tumor. This mass will eventually affect how an organ works. The original site of the abnormal cell growth is called the cancer’s “primary site”. In adrenal cancer, the primary site is the adrenal gland. Abnormal or uncontrolled cells can also grow and invade nearby organs and structures. This spreading is called metastasis. Tumors that invade and spread are called malignant or cancerous, while benign adrenal tumors never spread or invade other organs. ACC has often invaded nearby tissues or metastasized to distant organs at the time of diagnosis.  Most adrenocortical cancers are found after they have been growing for years. Fewer than 30% of adrenocortical cancers are confined to the adrenal gland at the time of diagnosis.

The most common symptom reported by patients with adrenocortical cancer is pain in the back, side, or stomach. Unfortunately, this type of pain is common and does not directly suggest a disease of the adrenal cortex. In adrenocortical cancer, these symptoms usually are due to pressure caused by the tumor as it compresses organs, nerves and other structures around the adrenal gland. Some patients describe feeling full with no appetite because of pressure on the stomach and other abdominal organs.

ACC is diagnosed through imaging (CT, MRI, unltrasound, PET) and confirmed by pathology of tumor.  Blood and urine tests may also be done.

The following system of staging for ACC is the most recently developed and implemented by the World Health Organization (WHO).

Stage 1:

The cancer is smaller than 5 centimeters (2 inches) and has NOT spread outside of the adrenal gland

Stage 2: 

The cancer is larger than 5 centimeters and has NOT spread outside of the adrenal gland

Stage 3: 

The cancer has spread into the fat surrounding the adrenal gland or has spread to lymph nodes or other organs near the adrenal gland.

Stage 4: 

The cancer has spread to adjacent organs other parts of the body

In general, adrenal cancers confined to the gland should be surgically removed. Those with spread to nearby or distant organs may be treated with surgery along with other therapies, or may be ineligible for surgical removal. The prognosis is best when the cancer can be surgically removed. Adrenal cancer is an aggressive cancer and the only known cure is complete surgical removal of the cancer, which may include removal of the adrenal gland as well as any other surrounding organs that the cancer invaded.

Chemotherapy is the treatment of cancer with drugs that can destroy cancer
cells.  These drugs destroy cancer cells (and harm healthy cells which usually repair themselves) by stopping them from growing or multiplying. Chemotherapy is the treatment of choice for adrenal cancer that cannot be cured by surgery. This stage of adrenal cancer is usually metastatic (spread to other organs). The goals of chemotherapy treatment are to control the cancer, keep it from spreading by slowing the cancer’s growth and improve or reduce the symptoms of the disease.  Some of the chemotherapy drugs that have shown some success against ACC are mitotane, streptozotocin, etoposide, doxorubicin and cisplatin.  In general, adrenocortical cancer can be aggressive and resistant to most chemotherapy. Most studies have demonstrated response rates of 30% to 50%. Studies of newer chemotherapy agents are ongoing and patients should also consider participating in a clinical trial (a research study or protocol) for the treatment of adrenal cancer. 

Radiation therapy (use of a beam of energy called ionizing radiation to kill cancer cells and shrink tumors) is generally not a standard treatment for ACC. It can be used for certain circumstances such as treatment after surgery to decrease the risk of local recurrence; or managing/controlling areas of tumor metastasis. This is useful in treating bone involvement. 

The best prognosis for adrenocortical cancers is seen in patients whose cancer is in Stage I or II. This means their cancer is confined to the adrenal gland and can be removed with surgery. The 5 year overall survival rate, which means the number of patients who will be alive 5 years after cancer treatment, is 40-60%. Stage III cancers have an overall survival rate of 20%. Stage IV cancers overall survival rate is 10%.  Prognosis is better in younger patients, while hormone secreting tumors are associated with worse prognosis.

 

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