The Basics of Adrenocortical Carcinoma

The adrenal glands are two small triangular-shaped organs resting atop the kidneys. These glands are responsible for producing hormones that are essential in controlling different functions in the body.

Each adrenal gland has two parts – the cortex and the medulla. The adrenal cortex produces hormones that control blood pressure as well as balance salt and water levels in the body. It is also responsible in the usage of protein, fat, and carbohydrates. In addition, it is also in charge of how feminine or masculine characteristics are developed.[1]

The adrenal glands perform important functions to ensure our bodies are working properly. Moreover, like any other organ, it is not immune to diseases, especially cancers such as Adrenocortical Carcinoma.

What is Adrenocortical Carcinoma?

Adrenocortical Carcinoma (ACC) is a rare and aggressive disease characterized by abnormal cell growths in the adrenal cortex (the outer layer of the adrenal glands) affecting only 0.72 persons per a million in population, according to The National Cancer Institute.[2]

Adrenocortical Carcinoma is classified in two types – Functioning and Non-functioning tumors. Functioning tumors are those that increase hormone production. Excessive amounts of testosterone, cortisol, and aldosterone will be produced with this type of Adrenocortical Carcinoma. Non-functioning tumors, on the other hand, do not increase hormone production, but are threatening nonetheless.[3]

Causes of Adrenocortical Carcinoma

The direct causes of adrenocortical carcinoma are unknown, but medical experts believe genetic predisposition greatly affect its development. Some 15% of Adrenocortical Carcinoma cases (mostly in children) are found to be due to a genetic defect, but all others are considered sporadic or those that only develop as a result of other risk factors experienced by a person within his lifetime.

The Li-Fraumeni Syndrome is one of the most common risk factors associated with Adrenocortical Carcinoma. It is a rare condition characterized by a TP53 gene defect. This condition not only heightens the risk of a patient developing Adrenocortical Carcinoma, but also cancers of the breast, bone, and even brain.

Smoking and other hazardous lifestyle habits also increase the risk of developing Adrenocortical Carcinoma although none has been proven definitively.[4]

Symptoms of Adrenocortical Carcinoma

It is rather difficult to diagnose Adrenocortical Carcinoma in its early stages as it rarely exhibits obvious symptoms. Functioning tumors that result in the increase of cortisol and aldosterone lead to symptoms such as:

A series of tests is required to ascertain that a patient is indeed suffering from Adrenocortical Carcinoma.[5]

Possible treatment of Adrenocortical Carcinoma

Treatment for Adrenocortical Carcinoma depends on the severity of the case. Surgical removal of the affected adrenal gland is the best treatment available to remove the diseased adrenal gland as well as any surrounding tissues affected by cancer cells. Adjuvant therapy is given to kill any cancer cells left following the surgery. Radiation and Chemotherapy may also be prescribed afterwards to reduce the risk of Adrenocortical Carcinoma coming back.[6]


[1] Adrenocortical Carcinoma Treatment (PDQ®)–Patient Version. National Cancer Institute. (Updated 2018 May17).

[2] Adrenocortical Carcinoma Treatment (PDQ®)–Health Professional Version. National Cancer Institute (Updated 2018 October 19).

[3] Adrenal Cortical Carcinoma. Healthline.

[4] Adrenal Cancer Risk Factors. American Cancer Society.

[5] Adrenocortical Carcinoma. MedlinePlus (Updated 2018 December 6).

[6] Treatment Choices by Stage of Adrenal Cancer. American Cancer Society.

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