What is Adrenal Cancer?

Adrenal cancer takes place in the adrenal glands which are located just above the kidneys. The adrenal glands are responsible for creating hormones. There are two main types of adrenal cortical tumors: benign  tumors and malignant tumors. Benign tumors in the adrenal gland are called adenomas and malignant tumors are called adrenal cortical carcinomas. Benign tumors do not spread cancer and can be removed through surgery. They do, however, have the ability to cause hormonal complications. Benign tumors in the adrenal gland may secrete an excess of hormones that cause Cushing's or Conn's syndrome. Malignant tumors in the adrenal glands have the ability to spread cancer cells to other sites in the body. When adrenal cancer has time to advance in staging, the results are often deadly. Malignant tumors need to be removed through a surgery called an adrenalectomy and treated with the chemotherapy drug mitotane.

Adrenal Cancer Causes

The direct cause of adrenal cancer is not known. While cell mutations are responsible for causing the disease, researchers do not know what causes a person's cells to mutate. Some cell mutations could be inherited from the DNA instructions of a parent, but only a small percentage. Outside variables such as radiation and carcinogens in the air could possibly cause cell mutations as well. It is believed that most adrenal cancer causing cell mutations occur after birth for no apparent reason at all. Not knowing a direct cause for adrenal cancer makes the disease difficult to prevent and near impossible to see coming before it is diagnosed.

Adrenal Cancer Prevention

Adrenal cancer cannot be prevented. Researchers have not yet found a way to prevent the cell mutations that cause adrenal cancer. Also, spending money, time and research to stop something that only might happen because a parent had DNA that caused cell mutations is not realistic. The best advice that can be given to prevent death caused by adrenal cancer is to avoid radiation and carcinogens and be screened at the doctor to check for adrenal cancer signs. Catching the disease in its earliest stage gives patients the best chance of surviving adrenal cancer.

Types of Adrenal Cancer

Adrenal cancer can exist in two different places in the adrenal gland, the adrenal cortex, and the adrenal medulla. These two areas can create two types of tumors each:

Adrenal Cortex

Adrenocortical adenoma tumors are benign and quite common. They do no cause or spread cancer. Some of them cause hormonal problems as a result of Cushing's and Conn's syndromes.

Adrenocortical carcinoma tumors are malignant, aggressive, and highly functional. These tumors grow quickly and are usually not discovered until they are in their more advanced stages. The prognosis for these tumors is very poor.

Adrenal Medulla

Neuroblastoma tumors are aggressive and usually occur in young children. Despite the ability of these tumors to spread to the liver, skin and blood, neuroblastomas are highly treatable. Surgery can be performed to remove the tumors and radiation and chemotherapy can treat neuroblastomas that have metastasized.

Pheochromocytoma tumors are malignant only 10% of the time. Pheochromocytomas can produce dangerous levels of adrenaline resulting in life-threatening high blood pressure. Other symptoms that may result from dangerous levels of adrenaline being released are headaches, anxiety, and panic attacks. Treatment options include surgery to remove the tumor and anti-adrenergic drugs.

Adrenal Cancer Symptoms

Adrenal cancer has different sets of symptoms depending on if the androgen or estrogen symptoms take place. Androgen and estrogen symptoms can occur in either males or females. Symptoms are usually more evident in children than in adults because adults have already gone through so many hormonal changes. Since symptoms show themselves more in children, children have a better chance of being diagnosed with adrenal cancer earlier than adults, thus giving young people a better chance of successfully treating the disease.

Androgen symptoms that can effect both male and female children include:

Excess growth of facial, pubic and underarm hair

Males will have an enlargement of the penis

Females will have an enlargement of the clitoris

Estrogen symptoms that can effect both male and female children include:

Early puberty in girls resulting in periods at a young age and enlarged breasts

Males may experience enlarged breasts

Adrenal Cancer Diagnosis

An adrenal cancer diagnosis can be made after a series of tests have been completed. A diagnosis cannot be made through only discussions and symptoms. There are three key areas of testing that contribute to diagnosing adrenal cancer. Those areas of testing are imaging tests, blood and urine tests and physical tests.

Imaging Tests Include

X-rays

CT Scans

MRI Scans

Ultrasounds

Blood and Urine Tests Include

High Cortisol Level Tests

High Aldosterone Level Tests

High Androgen and Estrogen Level Tests

Physical Tests Include

Biopsy

Laparoscopy

Adrenal Cancer Staging

Adrenal cancer stages are classified using the model created by the American Joint Committee on Cancer. The AJCC uses a TNM model as part of the staging system for adrenal cancer. The letters "T", "N" and "M" refer to the tumor, lymph nodes and metastasizing of the cancer. Numbers or letters are placed next to each letter to characterize how severe each aspect of the TNM model is. Higher numbers for each letter may mean a higher stage is needed to describe the current stage the adrenal cancer is in.

Five-year survival rates for early stages are relatively favorable at 65%. For adrenal cancer, stages 1 and 2 are considered early stages. The third and fourth stages have 40% and 10% five-year survival rates, respectively. These percentages are not based on a large sample of people, as adrenal cancer is a rare disease.

Adrenal Cancer Prognosis

Adrenal cancer prognoses depend on the type of tumor a patient has along with their age and past medical history. The stage a tumor is in is the most important factor for determining a prognosis. Benign and malignant tumors have an over all 65% five-year survival rate after removal. Malignant tumors alone have a five-year survival rate at about 40% after removal of a tumor. If a tumor is able to reach the fourth stage in it's progression, patients only have about a 10% chance of living at least five years.

Adenocortical carcinomas present the worst prognosis of any adrenal gland tumors. Most adrenocortical adenoma and pheochromocytoma tumors are benign offering a positive prognosis. Neuroblastomas are rare in that they can spread to distant sites in the body but can easily be treated through surgery and chemotherapy resulting in a positive prognosis.

Adrenal Cancer Treatment

Treatment options for adrenal cancer are limited to surgery to remove cancer cells and chemotherapy to minimize symptoms suffered as a result of hormonal imbalance. Surgery to remove cancerous tumors and cells from the adrenal glands can either be performed as an open surgery or by a laparoscopy.

Surgery

Open surgery involves a large incision across the belly or just below the rib through a patient's back. This is an invasive surgery that will require a long hospital stay. Any organs that have been infected with cancer will have to be removed though the belly. Large tumors are removed through open surgery.

Laparoscopy is a procedure characterized by three to five small incisions through a patient's abdomen. A tiny video camera is attached to the doctor's tools and this offers the doctor the best view possible inside the body. The majority of laparoscopies remove benign, small tumors. A laparoscopy offers a faster recovery time than open surgeries.

Chemotherapy

Chemotherapy is not used to cure adrenal cancer. It is used to control hormonal symptoms patients may experience. Even benign tumors can cause Cushing's and Cronn's syndromes. Those symptoms result in an excess of hormones to be secreted from the adrenal glands. Mitotane, a chemotherapy drug, help to reduce the symptoms suffered as a result of tumors in the adrenal glands. Mitotane can also be used to prevent cancer from reemerging and stopping it from spreading beyond the adrenal glands.

Adrenal Cancer Research

Research on adrenal cancer is difficult to attain because the disease is very rare, offering few subjects to study. Funding for clinical trials is low because adrenal cancer makes up for only 0.2% of cancer related deaths. Gene therapy methods to control genes and prevent them from causing adrenal cancer are being studied on animals, not humans. New chemotherapy drugs are being researched to find out if they can be more effective than mitotane at killing cancer cells in the adrenal glands.

Three major funds have been created to collect donations for adrenal cancer research:

The Millie Schembechler Adrenal Cancer Fund was created by Bo Schembechler after his wife Millie died of the disease in 1992. It is one of the largest adrenal cancer organizations and it offers an endowed professorship.

The Garry Betty Cancer Fund was created by former EarthLink CEO Garry Betty. He was diagnosed with adrenal cancer in 2006 and started the organization off with a $400,000 donation. The organization pays for international researchers to train in the field of adrenal cancer research.

The Spencer Bell Memorial Fund and Scholars Program offers sponsorships to undergraduate and graduate students to take part in internships. The internships are based on laboratory science aimed at adrenal cancer research.