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Adrenocortical Carcinoma - An Overview

There are over 200 types of cancer. Breast, prostate, and bowel cancers are the most common, while kidney, skin, and pancreatic cancer are less common. Then there are the rare cancers, like adrenocortical carcinoma (ACC). Also known as cancer of the adrenal cortex, ACC is a tumor that develops on the outer layer of the adrenal gland. Only one in one million people has it, and it’s more prevalent among women than men.

Adrenal glands are the triangle-shaped organs that sit on top of each kidney and produce hormones that give the body feminine or masculine features. They also help balance blood pressure and water and salt in the cells. As such, the growth of a tumor in the adrenal gland impairs the gland’s ability to regulate key bodily functions.

An ACC tumor may be functioning or non-functioning. A functioning ACC tumor causes the overproduction of hormones like testosterone, cortisol, or estrogen. A non-functioning ACC tumor does not impair hormone production.

Due to the nature of the hormones impacted by functioning ACC tumors, ACC affects men and women differently. Overproduction of testosterone in women, for instance, may cause acne, no menstrual periods, balding, and hair growth on the face. Too much estrogen in men may lead to impotence, lower sex drive, and enlarged breasts. Overproduction of cortisol may lead to high blood sugar, weight gain, or high blood pressure.

Half the time, ACC is genetic. People with close relatives who have ACC run the risk of developing it. Hereditary conditions associated with ACC include Li-Fraumeni syndrome, which impairs the TP53 gene responsible for suppressing tumors. Beckwith-Wiedemann syndrome (BWS) is a growth disorder at birth that causes abnormally large body parts.

Because of ACC’s impact on blood sugar levels, urine and blood tests may be used to diagnose it. Imaging studies, such as CT (computed tomography) scan or MRI (magnetic resonance imaging), may also be used. Adrenal angiography or adrenal venography may be conducted to monitor blood flow near the adrenal arteries and veins. A biopsy may also be done, which involves extracting a small tissue and studying it under a microscope.

Certain factors determine treatment options and chances of recovery (prognosis). Factors such as organs affected, whether the cancer has been treated in the past, the patient’s general health, and cancer stage to determine the mode of treatment. Stage I ACC may be treated by removing one or both adrenal glands, while Stage IV may be treated through radiation therapy, chemotherapy, and surgery.

Generally, the earlier a cancer is detected, the better the prognosis. Early diagnosis and treatment prevent the cancer cells from multiplying and spreading to other body parts (metastasis). If ACC spreads to the lung, the resultant cancer would be called metastatic ACC, not lung cancer.

Unlike common cancers, whose causes are often diverse, caused by an array of mutations, and driven by various biological and environmental factors, rare cancers are often more uniform. This makes it relatively easier to identify the most relevant causes and factors that sustain the tumors. Rare cancer research is vital to uncovering the biological, clinical, and molecular features of subsets of common cancers.