Adrenocortical Carcinoma

What is adrenocortical carcinoma?

Adrenocortical carcinoma is a cancer of the adrenal gland. The condition is also called cancer of the adrenal cortex, adrenal cortical cancer, or adrenocortical cancer. This cancer is very rare.

There are two adrenal glands in the body. One sits on top of each kidney. Adrenal glands are part of the endocrine system, which helps regulate body activities.

Adrenocortical carcinoma develops in the outer layer of the adrenal gland. This layer is called the cortex. It produces hormones that

help control blood pressure
balance water and salt in the body
help manage the body's use of protein, fat, and carbohydrates
contribute to a person having male or female characteristics.

Adrenocortical cancer can spread to other parts of the body including the lungs, liver, or bones.

Small lumps on the adrenal gland are not uncommon. They are usually benign (noncancerous) growths called adrenal adenomas. In some cases, doctors may have trouble distinguishing an adenoma from a cancer, so you may need special testing or repeat examinations.

Another type of adrenal gland tumor, called a pheochromocytoma, can develop in the inner part of the gland, or adrenal medulla. However, this article will only discuss cancers that arise in the adrenal cortex.

Symptoms of adrenocortical carcinoma

Tumors of the adrenal gland are classified as either functioning or nonfunctioning:

A functioning adrenocortical tumor overproduces certain hormones. It may trigger symptoms.
A nonfunctioning adrenocortical tumor doesn't secrete hormones. It may not cause symptoms early on.

The symptoms associated with functioning adrenocortical tumors vary. They depend on which hormones are overproduced and on the patient's age.

Cortisol helps the body use sugar, protein, and fats. Having too much of this hormone may cause

weight gain in the face, neck, chest, and abdomen
excessive hair growth on the face, chest, back, or arms (in women)
purple or pink stretch marks on the belly
a lump of fat behind the neck and shoulders
deepening of the voice
swelling of the sex organs or breasts (in men and women)
irregular menstrual periods (in women)
severe fatigue and muscle weakness
easy bruising
bone fractures
moodiness and/or depression
high blood sugar
high blood pressure.

Some of these symptoms are also characteristic of Cushing syndrome. This noncancerous condition affects the pituitary gland in the brain. Some, but not all, cases of Cushing syndrome are caused by an adrenal cancer or an adrenal adenoma.

Aldosterone helps control the balance of water and salts in the kidney. Having too much of this hormone may lead to

high blood pressure
muscle cramps or weakness
frequent urination
excessive thirst
fluid build-up in the body's tissues.

Androgens and estrogen are hormones that help develop and maintain sex characteristics that distinguish males and females. The major androgen is testosterone.

Women with too much testosterone may have

hair growth on the face and body
acne
balding
deepened voice
menstrual changes.

Women who have too much estrogen may experience

irregular menstrual periods
menstrual bleeding (if they have gone through menopause)
weight gain.

Men with too much estrogen may have

breast growth and tenderness
lower sex drive
inability to get or maintain an erection.

In children, the symptoms are different. An adrenal tumor that produces male sex hormones can cause

excessive facial, pubic, and underarm hair
an enlarged penis (in boys)
an enlarged clitoris (in girls).

A tumor that secretes female sex hormones in children can set off

early puberty (in girls)
breast enlargement (in boys).

Some patients experience symptoms when the tumor grows and presses on nearby organs and tissues. This may be felt as a lump or pain near the tumor, fullness in the belly, or trouble with eating or weight loss.

If you or your child has these symptoms, discuss them with your doctor right away.

Diagnosing adrenocortical carcinoma

The first step in diagnosis is usually a physical exam and medical history. Your doctor will check your body for signs of disease. He or she will want to know whether your family has a history of adrenal cancer. You will be asked about your sexual function and menstrual periods (if you are female).

A number of different tests and procedures may help diagnose adrenocortical carcinoma. They vary depending on the person's symptoms. You may have one or more of the following tests:

Blood and urine tests: Your blood and urine are analyzed to look for abnormal levels of hormones and other substances.
Imaging tests: Several types of imaging tests look inside the body for adrenocortical carcinoma. They can also help determine whether the cancer has spread to other parts of the body.
Adrenal angiography and venography: These tests show the arteries or veins and blood flow near the adrenal gland.
Biopsy: A needle is used to remove a piece of tumor that will be examined in a laboratory. The results can show where an adrenal gland tumor started. A biopsy can also confirm whether the cancer has spread. Needle biopsies are not usually the first tests performed on adrenal tumors, for several reasons. Performing a needle biopsy on an adrenal cancer can cause cancer cells to spread. Also, a biopsy may not be able to tell if an adrenal tumor is cancerous, because adrenal carcinomas (cancerous) and adrenal adenomas (benign) can look similar under the microscope. A biopsy of an area outside the adrenal glands can, however, confirm whether diagnosed adrenal cancer has spread.

If adrenocortical carcinoma is diagnosed, your care team will need to find out if the cancer cells have spread within or beyond the adrenal gland. This is called staging. Staging is important for planning treatment and assessing your prognosis. Many of the tests used for diagnosis also help doctors determine the stage of the cancer.

The four stages of adrenocortical carcinoma are defined by the size of the tumor and how far the cancer has spread:

Stage I: The tumor is 5 centimeters or smaller and is confined to the adrenal gland.
Stage II: The tumor is more than 5 centimeters and is found only in the adrenal gland.
Stage III: The tumor may be any size and may have spread to nearby tissues or lymph nodes.
Stage IV: The tumor is any size and has spread to nearby or distant sites.

Cancer that has returned after it has been treated is called recurrent adrenocortical carcinoma.

Expected duration of adrenocortical carcinoma

Adrenocortical carcinoma is hard to detect in its early stages. As a result, the tumor is often quite large when diagnosed. The disease tends to be caught earlier in children because they show signs of extra hormone production earlier than adults.

Even when adrenocortical cancer is removed completely by surgery, the disease often comes back or spreads to other parts of the body.

Preventing adrenocortical carcinoma

Most adrenal gland tumors are not related to family history. But some are linked to genetic defects. As a result, there is no known way to prevent the disease.

Treating adrenocortical carcinoma

Surgery is the treatment of choice for many adrenocortical carcinomas. However, your treatment will depend on your overall health and cancer stage.

Stage I and II: These cancers are generally treated by removing the diseased adrenal gland. Nearby lymph nodes may be removed for examination if they are enlarged. If the cancer is not producing hormones, additional treatment may not be needed. However, follow-up exams are recommended. If the cancer might not have been completely removed with surgery, your doctor may recommend more therapy with either radiation or the drug mitotane. Mitotane specifically attacks and kills adrenal cells.

Stage III: Surgery is the standard treatment for stage III adrenocortical carcinoma. The goal is to remove the affected adrenal gland. Nearby enlarged lymph nodes are also removed. Nearby organs may be removed as well. Patients with stage III adrenocortical carcinoma are at risk for recurrent cancer. Radiation and/or mitotane often follow surgery for stage III adrenocortical carcinoma.

Stage IV: Sometimes the cancer has spread and is not likely to be cured. Still, surgery may be recommended to remove as much of the tumor as possible. This can reduce symptoms and enhance the person's quality of life. Radiation therapy, chemotherapy, or immunotherapy may also help manage symptoms.

Recurrent adrenocortical cancer: This is usually treated like stage IV disease, but the approach depends on several factors. These include the patient's history with adrenocortical cancer and the site of the recurrence.

For stages III and IV and recurrent adrenocortical cancer, mitotane is often recommended with or without additional chemotherapy. Mitotane helps slow cancer growth and can decrease symptoms related to hormone overproduction.

When treatments are no longer helping, care will focus on controlling pain and improving well-being.

When to call a professional

Contact your doctor if you or your child has any symptoms of adrenocortical carcinoma, including

abdominal pain
unusual hair growth
irregular menstrual cycles
irregular sexual behavior
early puberty (in children).

Prognosis

The outlook for people with adrenocortical cancer depends on several factors. These include

the stage of the disease
whether the tumor can be removed completely with surgery
how quickly the cancer is growing
whether the cancer has been treated in the past
the patient's general health.

Patients with early stage adrenal cancer have a good outlook for survival. Many can be cured with surgery. Individuals with more advanced disease have a less favorable prognosis.

Additional info

National Cancer Institute (NCI)
https://www.cancer.gov/

American Cancer Society (ACS)
https://www.cancer.org/