Diseases of the Adrenal Cortex: Adrenal Cancer

Diseases of the Adrenal Cortex: Adrenal Cancer
James Norman, M.D., F.A.C.S.
endocrineweb

Symptoms, Diagnosis and Treatments
Adrenocortical carcinoma is a rare tumor afflicting only one or two persons per one million population. 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.

Characteristics of Adrenal Cortical Cancer
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.

Evaluation of a Suspected Adrenal Cortical Cancer
The Initial evaluation should include blood tests to measure the amount of adrenal hormones in the circulation. Since the vast majority of these cancers make too much hormone (cortisol, testosterone, estrogen, aldosterone, etc) this is an obvious place to start. Keep in mind, however, that most non-cancerous tumors of the adrenal glands (benign adenomas and hyperplasia) will also secrete too much hormones. Therefore, demonstrating overproduction of adrenal hormones helps establish the presence of an adrenal tumor, yet it does not always help distinguish between benign and malignant (cancerous) tumors. Extremely high levels, however, are more commonly produced by malignant tumors.

X-ray tests play a central role in the diagnosis of adrenal cancers, and undoubtedly will play a central role in determining the type of treatment planned. Computed tomography (CAT scans) and/or magnetic resonance imaging (MRI) are the two central tests in this regard. They give overlapping information, so that all persons do not need both tests, but occasionally the situation will dictate that both are obtained. The CAT scan on the right shows a large right adrenal mass outlined in yellow under the normal sized liver (in red). This tumor produced large amounts of estrogen and caused the patient (a 66 year old woman) to seek medical attention after she began to have menstrual bleeding 20 years after her menopause.

Some adrenal tumors will require special studies of their blood supply to help define the extent of the tumor, whether it is impinging on the blood supply to other nearby organs, and to help the surgeon decide on which operative approach to use. These tests are referred to as selective angiography and adrenal venography. They also may be helpful in distinguishing tumors of the adrenal gland from tumors of the upper pole of the kidney.

Adrenal Syndromes Caused by Excess Hormone Secretion
As noted in our example above, many patients will seek medical attention with some sort of bodily change which typically comes on quite slowly (usually over one to three years). When excess female hormones are produced in a female it can be hard to detect, except at extremes of age such as early puberty in a child, or the return of vaginal bleeding in a post-menopausal woman. The same is true for excess testosterone in a male. The opposite, however, will often make the presentation easier such as when a woman begins to develop male characteristics (deeper voice, excess body hair) or when a man begins to develop enlarged breasts. Some of these hormone overproduction problems have specific names and are listed below.

hypercortisolism (Cushing's syndrome) (excess cortisol produced)
adrenogenital syndrome (excess sex steroids produced)
virilization (acquisition of male traits in a female because of excess testosterone production)
feminization (acquisition of female traits in a male because of excess estrogen production)
precocious puberty (puberty occurring too early because of excess sex steroids produced)
hyperaldosteronism (Conn's syndrome) (excess aldosterone leading to hypertension and low potassium)

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