Adrenal cortical carcinoma (ACC) is a rare disease. It is caused by an increase in cancer in the adrenal cortex, the outer layer of the adrenal glands. The adrenal glands are located above the kidneys. They play an important role in the endocrine system, the system that produces and regulates hormones. ACC is also known as adrenocortical carcinoma.

The adrenal cortex produces hormones that regulate metabolism and blood pressure. It also produces male hormones called androgens like cortisol and testosterone. ACC stimulates a high production of these hormones.

Types of adrenal cortical carcinoma

There are two types of adrenal cortical carcinoma.

Functional tumors increase the production of adrenal hormones. With this type of tumor, large amounts of cortisol, testosterone, and aldosterone are commonly found in the body. (Aldosterone is a hormone that regulates blood pressure.)

Dysfunctional tumors do not increase the production of hormones by the adrenal glands.

Most tumors of the adrenal glands are not cancerous. Only 5 to 10 percent of adrenal tumors are malignant.

What are the symptoms of adrenal cortical carcinoma?

The symptoms of a functional tumor depend on the hormones it produces.

• Testosterone and other androgens:
• Increase in facial and body hair, especially in women.
• Deep voice in women
• Estrogen:
• The first signs of puberty in boys.
• Enlarged breast tissue in men
• Aldosterone:
• Weight gain
• Hypertension
• Cortisol:
• High blood sugar and pressure
• Muscle weakness in the legs
• Injury to the body
• Excess weight increases in the chest and abdomen.
  

Abdominal pain occurs if both functional and non-functional tumors have spread. Dysfunctional tumors do not cause any hormonal changes or cause specific symptoms.

Cushing's syndrome is a condition in which cortisol causes adrenal tumors. Although ACC causes Cushing, most tumors associated with this condition are benign. If you have Cushing, this does not mean that you have cancer.

Causes

Doctors are not sure what causes adrenal cancer. It begins with a change in the genes of some adrenal cells, but that change happens randomly.

It is hereditary, so if anyone in your family has cancer of the adrenal glands or other parts of the endocrine system, tell your Oncologist. If you can get it, regular checkups can help you catch up early.
When you have a genetic condition, you have a defect in one or more genes. Certain genetic conditions make you more susceptible to adrenal cancer:

• Beckwith-Wideman syndrome
• Familial adenomatous polyposis (FAP)
• Hereditary nonpolyposis colorectal cancer (HNPC)
• Li-Frameni syndrome
• Multiple endocrine neoplasia (MEN1)

Risk factors

Adrenal cancer happens more often in people with inherited syndromes that increase the risk of certain cancers. These inherited syndromes include:

• Beckwith-Wiedemann syndrome
• Carney complex
• Li-Fraumeni syndrome
• Lynch syndrome
• Multiple endocrine neoplasia, type 1 (MEN 1)

Diagnosis

Your doctors will start with questions about your symptoms, medical history, and cancer in your family. They will also do a physical exam; Your doctor will feel your abdomen for the tumor. From there, you will have different blood and urine tests to monitor your hormone levels; high levels may be a sign of a tumor, but it is not cancer.

Your doctor may also use:
Computed tomography. This is the most common picture to look for in adrenal gland tumors.

MRI may not be as widely used as CT scan, but it can help.

Laparoscopy. Your doctor uses a thin, flexible tube with a camera on the end, a laparoscope, that goes into an opening made in your side to see the cancer. Helps the doctor determine if surgery can remove the cancer.

Pet scan. This will help your doctor determine if the tumor is cancerous.

Because your adrenal glands are so close to certain vital blood vessels, you can also get an adrenal angiogram or adrenal venogram. These tests measure the blood flow in the arteries and veins around the adrenal gland.

Unlike other cancers, a biopsy may not be done before surgery if imaging tests and blood tests are adequate.

Treatment

Treatment for adrenal cancer usually involves surgery to remove the entire tumor. Other treatments may be used to prevent cancer recurrence or if surgery is not an option.

Surgery

The goal of surgery is to remove all of the adrenal cancer. To accomplish this, doctors must remove the affected adrenal gland (adrenalectomy).

If surgeons find evidence that the cancer has spread to nearby structures, such as the liver or kidneys, those parts or all of those organs can also be removed during the operation.

Medicines to reduce the risk of recurrence.

The old drug used to treat advanced adrenal cancer promises to delay the recurrence of the disease after surgery. Mitotane (lysodren) may be recommended after surgery for those at risk of cancer recurrence. Research on mitotane for this use is ongoing.

Radiotherapy

Radiation therapy uses high-energy rays, such as x-rays and protons, to kill cancer cells. Radiation therapy is sometimes used to destroy cells that remain after adrenal cancer surgery. It can also help reduce pain and other symptoms of cancer that has spread to other parts of the body, such as the bones.

Chemotherapy

Chemotherapy is the treatment of cancer by using chemicals to kill cancer cells. For adrenal cancers that cannot be surgically removed or come back after initial treatment, chemotherapy is an option to slow the progression of the cancer.