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The A To Z Of Adrenocortical Carcinoma

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The media fraternity and the nation at large are still mourning the death of former NTV news anchor year-old Rosemary Nankabirwa, who passed away on Sunday. The 37 year old beloved anchor succumbed to adrenocortical carcinoma: A rare cancer that forms in the outer layer of tissue of the adrenal gland (a small organ on top of each kidney that makes steroid hormones, adrenaline, and noradrenaline to control heart rate, blood pressure, and other body functions).   It produces hormones that cause changes such as weight gain and fluid retention, early puberty in children, or excess facial or body hair growth in women.

It starts causing symptoms because it has gotten very large. Large tumors can press on other organs in the abdomen, causing pain or a feeling of fullness. Generally, adrenal cancers are much larger than adrenal adenomas. An adrenal tumor larger than 5 or 6 centimeters (about 2 to 2 1/2 inches) is assumed to be a cancer. In one study, the average size of an adrenal cancer was about 13 cm (or 5 inches).

Most cancers found in the adrenal gland did not start there and are not adrenal cancers. Instead, they start in other organs or tissues and then spread (metastasize) through the bloodstream to the adrenal glands. For example, lung cancers, melanomas, and breast cancers often spread to the adrenals. Even when other cancers spread to the adrenals; however, they are still named after the place they started and are treated like other cancers that start in the same place. They are not considered adrenal cancer. Their treatment is described in our documents on these cancers.

Statistics

Adrenal carcinomas are very rare and the real number is placed at around 200 per year. They are much less common than benign adrenal tumors (adenomas), which are found fairly often among middle aged and elderly people. Adrenal tumors (most of which are adenomas) are found in about one in every 10 people who have an imaging test (like a CT or MRI) of the adrenal gland. The average age of patients with adrenal cancer is around 46, but adrenal cortical cancer can occur in people of any age; even in children.

Risk factors

A risk factor is anything that changes your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed.

Scientists have found few risk factors that make a person more likely to develop adrenal gland tumors. Even if a patient does have one or more risk factors for adrenal gland tumors, it is impossible to know for sure how much that risk factor contributed to causing the cancer.

 What causes adrenal cancer?

Causes of most adrenal cortical tumors are still unknown. Over the past several years, experts have made great progress in understanding how certain changes in a person’s DNA can cause cells in the adrenal gland to become cancerous. Cancers can be caused by DNA mutations (changes) that turn on oncogenes or turn off tumor suppressor genes. Some people with cancer have DNA mutations they inherited from a parent, which increase their risk for developing the disease. But most DNA mutations that are seen in cancers happen during life rather than having been inherited. These mutations may result from exposure to radiation or carcinogens (cancer-causing chemicals). But most of these mutations happen for no apparent reason.

Prevention

Since there are no known preventable risk factors for this cancer, it is not now possible to prevent this disease, specifically. Not smoking is a way to lower the risk for many cancers, and perhaps even adrenal cortical cancer.

Lifestyle and environmental factors

Risk factors such as a high-fat diet, smoking, sedentary lifestyle, and exposure to cancer-causing substances in the environment have a great impact on a person’s risk of developing many types of cancer. Although none of these factors has been definitely found to influence a person’s risk of developing adrenal cancer, smoking has been suggested as a risk factor by some researchers.

Genetic syndromes

The vast majority of adrenal cortex cancers are sporadic (not inherited), but some (up to 15%) are caused by a genetic defect. This is more common in adrenal cancers in children.

Detection

It is hard to find adrenal carcinomas early and they are often quite large when diagnosed. Adrenal carcinomas are often found earlier in children than in adults because adrenal cancers in children more commonly secrete hormones. Children will show outward signs of excess hormone production early. For example they may develop very early signs of puberty due to the sex hormones that sometimes are produced by adrenal cancer. In adults, these tumors may be found early by accident, when a CT (computed tomography) scan is done for some other health concern.

Signs and symptoms of adrenal cancers

The most common symptoms are excessive growth of facial and body hair (such as in the pubic and underarm areas). Male hormones may also enlarge the penis in boys or the clitoris in girls.

If the tumor secretes estrogens (female-type hormones), girls can start puberty early. This can cause the breasts to develop and menstrual periods to start. Estrogen-producing tumors also may enlarge breasts in boys.

Weight gain, usually greatest above the collar bone and around the abdomen

Fat deposits behind the neck and shoulders

Purple stretch marks on the abdomen

Excessive hair growth on the face, chest, and back in women

Menstrual irregularities

Weakness and loss of muscle mass in the legs

Easy bruising

Depression and/or moodiness

Weakened bones (osteoporosis), which can lead to fractures

High blood sugar, often leading to diabetes

High blood pressure

As an adrenal cancer grows, it presses on nearby organs and tissues. This may cause pain near the tumor, a feeling of fullness in the abdomen, or trouble eating because of a feeling of filling up easily.

 

 

 

 

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