Last Updated on Feb 07, 2016

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Adrenocortical carcinoma (ACC) is an incompletely understood rare malignancy with poor treatment outcome and prognosis.

Adrenocortical carcinoma is a rare but aggressive form of cancer that affects the outer layer (cortex) of the adrenal glands situated atop the kidneys. The cortex has several important functions such as producing adrenaline, nor adrenaline and steroid hormones besides controlling heart beat rate and blood pressure.

This condition affects about 1-2 million people annually; children below the age of 6 years and adults aged between 30-40 years are the most affected.

Adrenocortical Carcinoma

The adrenal is an important hormonal gland of the body and produces glucocorticoids, mineralocorticoids and sex hormones hence the cancer of this gland can cause excess production of these hormones leading to many syndromes depending on the cell type that proliferates. Sometimes the cell type that proliferates may not produce a functional tumor.

In children when it manifests as a cancer it is usually a functional tumor. Virilization is by far the most common presenting symptom, followed by Cushing's syndrome and precocious puberty.

Among adults presenting with hormonal syndromes, Cushing's syndrome alone is most common, followed by mixed Cushing's and virilization (glucocorticoid and androgen overproduction). Feminization and Conn syndrome (mineralcorticoid excess) occur in less than 10% of cases.

In adults the presentation maybe as a functional tumor or a non-functional tumor (almost in 40%). Among functional tumors the most common is Cushing's syndrome, followed by mixed Cushing's and virilization. Feminization and Conn syndrome (due to mineralcorticoid excess) occur in less than 10% of cases.

Like any cancer this can also often invade nearby tissues or spread to distant organs like bone, liver and lungs.

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