What is adrenocortical carcinoma?

Adrenocortical carcinoma (ACC) is a rare disease, caused by the development of a cancer of the adrenal cortex, which is the outer layer of the adrenal gland. The adrenal gland is located above the kidney. They play an important role in the endocrine system, the hormone production and regulation system.

Information about adrenal glands

The adrenal gland is a small organ above the kidney. It makes hormones important. There are two adrenal glands in the body. One sits on top of each kidney. The adrenal gland is part of the endocrine system. Your endocrine system will help regulate body activities.

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

• Helps control blood pressure
• balance water and salt in the body
• Helps manage the body’s use of proteins, fats, and carbohydrates
• makes a person masculine or feminine.

Adrenocortical cancer can spread to other parts of the body including the lung, liver, or bone.

Small lumps on the adrenal glands are not uncommon. They are usually benign (not cancerous) growths called adrenal adenomas. In some cases, your doctor may have difficulty distinguishing an adenoma from a cancer, so you may need to test or repeat a special exam.

A type of adrenal gland tumor, called pheochromocytoma, can develop in the inner part of the gland, or adrenal medulla. However, this article will only discuss cancer arising in the adrenal cortex.

Types of adrenocortical carcinoma

There are two types of adrenocortical carcinoma.

• Functional tumors increase adrenal hormone production. 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.)
• Non-functional tumors do not increase adrenal hormone production.

Most adrenal tumors are not cancer. Only 5 to 10% of adrenal tumors are malignant.

Symptoms of kidney adenocarcinoma

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

• An adrenal tumor activates certain hormone overproduces. It may cause symptoms.
• A nonfunctioning adrenal tumor does not secrete hormones. It may not cause symptoms early.

The symptoms associated with adrenal tumors vary. They depend on which hormone is overproduced and on the age of the patient.

Cortisol helps sugar use the body, protein, and fat. Too much of this hormone can cause

• weight gain in the abdomen, face, neck, and chest
• Excessive hair growth on face, chest, back, or arms
• Purple or pink stretch marks on the abdomen
• a lump of fat behind the neck and shoulders
• deep voice
• swelling of the genitals or chest (in men and women)
• irregular menstrual cycle (in women)
• Severe fatigue and muscle weakness
• easy bruising
• fracture
• moodiness and / or depression
• high blood sugar
• High Blood Pressure.

Some of these symptoms are also characteristic of Cushing’s syndrome. This non-cancerous condition affects the pituitary gland in the brain.

Aldosterone  helps control the balance of water and salt in the kidneys. Having too much of this hormone can result

• High Blood Pressure
• muscle weakness or cramping
• frequent urination
• feeling thirsty
• Fluid accumulates in the tissues of the body

Androgens and estrogens are hormones that help develop and maintain the gender identity that distinguishes men and women. The androgen hormone is testosterone. Women with too much testosterone may have

• Hair growth on face and body
• Acne
• bald
• A depth of voice
• menstrual changes.

Women with too much estrogen may experience it

• irregular menstrual cycle
• menstrual bleeding (if they have stopped having time).

Men with too much estrogen may have

• breast development and tenderness
• low libido
• Inability to get or maintain an erection.

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

• Excessive facial, pubic, and armpit hair
• an enlarged penis (in boys)
• an enlarged clitoris (in women).

A tumor that secretes female sex hormones in children can pose

• precocious puberty (in women)
• breast enlargement (in boys).

Some patients have symptoms as the tumor grows and presses on surrounding organs and tissues. This may be felt like a tumor or pain near the tumor, fullness in the abdomen, or trouble with eating or losing weight.

If you or your child has these symptoms, talk to your doctor right away.

What causes adrenocortical carcinoma ?

The cause of adrenocortical carcinoma is still unclear. However, adrenocortical carcinoma can also be a secondary cancer. Secondary cancer is a cancer that occurs when another type of cancer has spread to the adrenal glands.

Who is at risk for adrenal carcinoma?

Scientists have identified several risk factors for adrenocortical carcinoma. You may be at higher risk if you:

• is female
• are in the age of 40 to 50
• have a genetic disease that affects the adrenal glands
• There is another invasive form of cancer

Children under 5 years old are also at high risk for this condition. Remember, adrenocortical carcinoma is a rare cancer. Just because you have one or more risk factors does not mean you will have adrenocortical carcinoma.

Prevent

Most adrenal adenomas are not related to a family history. But some are related to genetic defects. Therefore, there is no known way to prevent this disease.

Treatment

Surgery is the treatment and option for many adrenocortical carcinomas. However, your treatment will depend on your general health and cancer stage.

Stages I and II:  These cancers are usually treated by eliminating adrenal glands. Nearby lymph nodes may be removed to check if they are enlarged. If the hormone is not produced, additional treatment may not be needed. However, the next exam is recommended.

Stage III  : Surgery is the standard treatment for stage III adrenocortical carcinoma. The goal is to remove the affected adrenal glands. Lymph nodes nearby are also deleted. Neighboring bodies may be removed as well. Patients with stage III adrenal carcinoma are at risk of recurrent cancer.

Stage IV:  Sometimes the cancer has spread and is unlikely to be cured. However, surgery may be recommended to remove as many tumors as possible. This can reduce symptoms and improve the person’s quality of life. Radiation and chemotherapy may also help manage symptoms  .

Recurrent adrenal cancer:  This is often treated as stage IV disease, but this method depends on many factors. These include the history of patients with adrenocortical cancer and the site of relapse.

For stage III and IV and recurrent adrenal cancer, a combination of chemotherapy including a drug called mitotane can help develop cancer and reduce hormone-related symptoms in production.

When treatment no longer helps, care will focus on pain control and welfare improvement.

Source Reference Adrenocortical carcinoma:

• Source mayoclinic.org article Adrenal cancer : https://www.mayoclinic.org/diseases-conditions/adrenal-cancer/symptoms-causes/syc-20351026 , updated October 9, 2019.
• Source en.wikipedia.org Adrenocortical carcinoma article : https://en.wikipedia.org/wiki/Pancreatic_cancer , updated September 9, 2019 .
• A reputable source thuoclp.com summarizes the article What is adrenocortical carcinoma? https://index-china.com/adrenocortical-carcinoma/ , updated 12/3/2020.