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Cancers of the adrenal gland are very rare. The adrenal gland is a hormone producing endocrine gland with two main parts, the cortex and the medulla. The main hormone of the adrenal cortex is cortisol and the main hormone of the adrenal medulla is epinephrine. When tumors develop in the adrenal gland, they secrete excess amounts of these hormones. A cancer that arises in the adrenal cortex is called an adrenocortical carcinoma and can produce high blood pressure, weight gain, excess body hair, weakening of the bones and diabetes. A cancer in the adrenal medulla is called a pheochromocytoma and can cause high blood pressure, headache, palpitations, and excessive perspiration. Although these cancers can happen at any age, most occur in young adults.

The adrenal glands are orange-colored endocrine glands which are located on the top of both kidneys. The adrenal glands are triangular shaped and measure about one-half inch in height and 3 inches in length. Each gland consists of a medulla (the center of the gland) which is surrounded by the cortex. The medulla is responsible for producing epinephrine and norepinephrine (adrenaline). The adrenal cortex produces other hormones necessary for fluid and electrolyte (salt) balance in the body such as cortisone and aldosterone. The adrenal cortex also makes sex hormones but this only becomes important if overproduction is present.

Adrenal gland disorders occur when the adrenal glands don’t work properly. Sometimes, the cause is a problem in another gland that helps to regulate the adrenal gland. In other cases, the adrenal gland itself may have the problem. The NICHD conducts and supports research on many adrenal gland disorders.

Adrenocortical carcinoma is a rare tumor afflicting only one or two persons per one million populations. It usually occurs in adults, and the median age at diagnosis is 44 years. Although potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the time of diagnosis. Because these tumors tend to be found years after they began growing, they have the opportunity to invade nearby organs, spread to distant organs (metastasize) and cause numerous changes in the body because of the excess hormones they produce.

Typically an aggressive cancer.

Most (~60%) are found because excess hormone production causes symptoms which prompt patients to seek medical attention.

Most (60-80%) actually secrete high amounts of one or more adrenal hormones.

Many will present with pain in the abdomen and flank (nearly all that don't present with symptoms of hormone excess will seek medical attention because of pain).

Spread to distant organs (metastasis) occurs most commonly to the abdominal cavity, lungs, liver, and bone.

Treatment for Adrenal glands

Treatment is aimed at removing the tumor by surgery. In some cases, this can be done by laparoscopy. Surgery is sometimes followed by chemotherapy and/or radiation therapy. Because the surgery removes the source of many important hormones, hormones must be supplemented following surgery.
Laparoscopic Adrenalectomy - in this procedure, a fiber optic scope is inserted through a tiny incision made by your doctor in your abdominal cavity. It is a less invasive procedure and therefore generally causes less pain than other approaches. Thankfully most adrenal cancers can be removed using this technique.

Chemotherapy

Can be helpful in inducing regression in primary and metastatic tumour size
Mitotane most commonly used drug
Glucocorticoid and mineral corticoid replacement is necessary in most cases to prevent adrenal insufficiency
In a few cases, chemotherapeutic agents may have some antitumour activity, e.g., cyclophosphamide, doxorubicin, and cisplatin.


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