Adrenal cancer is a rare disease that originates in the adrenal glands. The adrenal glands are located on top of the kidneys and consist of two parts that function separately. The first part is referred to as the cortex (outer layer). The second part is referred to as the medulla (inner area). Nearly all adrenal tumors are benign (non-cancerous) and therefore do not require treatment.

The most common type of adrenal cancer is called adrenocortical carcinoma. Adrenocortical carcinoma develops in the adrenal cortex and may produce symptoms related to increased hormone production, if it is a functioning tumor. Non-functioning adrenal cancer tumors do not produce hormones and may cause pain from pressure applied to the abdominal organs.

A second type of adrenal cancer is called pheochromocytoma which is an extremely rare type of cancer that originates in cells that produce epinephrine and norepinephrine. This form of adrenal cancer is most prevalent among those in their 30's and 40's.

Neuroblastoma is the third type of adrenal cancer. This adrenal cancer develops in the adrenal medulla and typically originates in undeveloped nerve cells. Abdominal pain and bone pain are usually symptoms experienced with neuroblastoma. This type of cancer generally appears in infants and children.

Approximately 1 out of 1 million people worldwide develop adrenal cancer. What causes adrenal cancer is unknown. It is believed that sometimes heredity plays a role, so if there is a diagnosis of adrenal gland cancer in your family, you may want to consider genetic testing.

Causes adrenal cortical cancers ?

Each year, there are approximately 500 cases of adrenal cortical cancers in the United States. These most commonly occur in patients between the ages of 30 and 50; however, children under the age of 5 develop adrenal cortical cancers at a higher rate than the rest of the population. Males are more likely to develop non-functioning adrenal carcinomas, while females are more likely to develop functioning adrenal carcinomas. In general, causes of adrenal cortical cancers are unknown. They are not associated with smoking, and do not run in families. Despite this, certain genetic mutations have been associated with adrenal cortical cancers, and research is ongoing in attempt to identify the causes of these cancers.

Adrenal cancer - Signs and Symptoms

Although highly unlikely, because those diagnosed rarely exhibit symptoms, it is possible to experience the following:

• Fever
• Abdominal mass
• Weight loss
• Abdominal pain
• Always feeling full

Syndromes Associated with Adrenal Cancer

If you have a functioning adrenocortical tumor that produces excess cortisol, it could develop into Cushing's syndrome. Cortisol, also referred to as hydrocortisone, is the main circulating steroid hormone of the cortex. Cushing's syndrome is a hormonal disorder that is caused by prolonged exposure to high levels of cortisol. Cortisol performs vital tasks in the body such as maintaining blood pressure and controlling the function of your cardiovascular system. In addition, it makes fatty acids available for metabolic use.

Adrenal cancer symptoms associated with Cushing's syndrome include:

• Severe acne
• High blood pressure
• Weakness
• Hirsutism
• Amenorrhea
• Slowed growth rate in children
• Osteoporosis
• Abdominal stretch marks

Another syndrome that can develop is called Conn's syndrome. Conn's syndrome is a disease that involves excessive production of aldosterone. Aldosterone is a steroid that is secreted by the adrenal gland. It regulates your electrolyte balance and is considered the most potent hormone. Conn's syndrome is caused by increased aldosterone production.

Adrenal cancer symptoms associated with this may include:

• Low potassium levels
• High blood pressure
• Frequent urination
• Excessive thirst

Adrenal Cancer Treatment Options

There are various treatment options for adrenal cancer. One of the most commonly used treatment options for adrenal cancer is surgery. There are four types of surgery that can be performed to remove both benign and malignant adrenal cancer tumors. The procedure your doctor will use will depend on the type and size of the tumor and also your individual needs.

• Posterior Surgery - typically used to remove small, benign tumors, your doctor will make an incision in your back just above the kidneys.
• Transabdominal Surgery - in this procedure, an incision is made in your abdomen so the doctor can inspect nearby blood vessels and organs for any signs of cancer. It also allows your doctor to remove any nearby tissue that might be directly involved with the tumor.
• Thoracoabdominal Surgery - this is usually the procedure of choice if you a large, malignant adrenal tumors. Your doctor will make a large incision around the cancerous area so he/she has an expanded view of the tissue that surrounds the tumor.
• 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.

Radiation therapy is also a treatment option for some adrenal cancer patients. The high-energy rays used in radiation therapy destroy cancerous cells and reduce the tumor's size. It is most often utilized for adrenal cancer that has already started to spread. It is also a treatment that be used in conjunction with chemotherapy.

Adrenal Cancer Treatment at CTCA

The doctors and caregivers at Cancer Treatment Centers of America know how emotionally devastating a diagnosis of adrenal cancer can be, and make it our goal to ensure that you are as comfortable and informed as possible during this difficult time. We work with you to enrich your adrenal cancer treatment by not only offering the most up-to-date traditional treatments, but also by complementing them with new therapies designed to treat adrenal cancer using a whole-body approach.

At CTCA you and your healing are at the center of our hearts, minds and actions every day. If you are looking for an adrenal cancer treatment center that treats more than just your cancer, Cancer Treatment Centers of America may be exactly what you are looking for. To learn more about our integrated approach to cancer treatment, contact us today at 800-931-0599 and speak with one of our Oncology Information Specialists. They can help you decide if CTCA is the right treatment facility for you.

More Information about Adrenal Cancer

What are the adrenal glands?

The adrenal glands are small glands that are located just above each kidney (they are sometimes called the suprarenal glands for that reason). They are triangular in shape and consist of several distinct parts:

The central part of the gland is called the adrenal medulla and produces the chemicals epinephrine (also called adrenaline) and norepinephrine. Both of these chemicals are involved in regulation of the nervous system. Epinephrine controls the short-term stress response (aka fight-or-flight response). While norepinephrine also plays a role in short-term stress response, it functions in regulating mood and attention, as well.

The outside part of the gland surrounding the medulla is the adrenal cortex. This part of the adrenal gland is largely responsible for producing steroid hormones in the body. There are several types of steroid hormones that are produced by the adrenal glands. Mineralocorticoids (such as aldosterone) are steroid hormones that help regulate the salt levels in the body by controlling the absorption and excretion of salt and water in the kidneys. This in turn helps to regulate blood pressure. Glucocorticoids (such as cortisol) are steroid hormones that play a critical role in the regulation of sugar within the body. These hormones also help to regulate the fat stores within the body, act as a strong anti-inflammatory force, and play an important role in fetal development, particularly in lung maturation. The adrenal cortex also produces several sex steroid hormones, including androgens (critical for male sexual development) and precursors to estrogen (critical for female sexual development).

What are adrenal tumors, and what types of adrenal tumors are there?

Normally, cells in the body will grow and divide to replace old or damaged cells. This growth is highly regulated, and once enough cells are produced to replace the old ones, normal cells will stop dividing. Tumors occur when there is an error in this process, and cells continue to grow in an uncontrolled manner. Tumors can either be benign or malignant. Although benign tumors can grow in an uncontrolled way, they do not spread to other parts of the body (metastasize), nor do they invade surrounding tissues. Malignant tumors (also known as cancers) will grow uncontrolled in such a way that they invade and damage other tissues around them. They also gain the ability to break off from where they start and spread to other parts of the body, usually through the blood stream or through the lymphatic system where the lymph nodes are located (a process known as metastasis).

The most common tumor of the adrenal gland is actually a benign tumor called an adrenal adenoma. In most patients, these benign tumors never cause a patient to have any symptoms and do not need to be treated. They are usually found when a patient has a CT (or CAT) scan of the body for an unrelated reason, and are thus sometimes called “incidentalomas”. The most common malignant tumors found in the adrenal gland are tumors that come from cancer cells that have metastasized (or spread) from other parts of the body to the adrenal gland through the blood stream. Several different types of cancer may spread to the adrenal glands, most commonly melanomas, lung cancers, and breast cancers. The adrenal glands are the fourth most common site in the body for cancer cells to metastasize to, after the lungs, liver, and bone.

Cancers can arise directly within the adrenal glands themselves; however, these are relatively rare. Cancers may arise directly from the adrenal cortex, and are called adrenal cortical cancers. These cancers can either be functioning (meaning they secrete excess steroid hormones) or non-functioning (meaning they do not secrete steroids). Functioning adrenal cortical cancers are more common than non-functioning cancers. Cancers can also arise within the adrenal medulla, the most common of which are pheochromocytomas. In children, neuroblastoma tumors can develop within the adrenal medulla. Pheochromocytomas and neuroblastomas are discussed elsewhere individually, will not be discussed further in this review.

Other types of adrenal cancers can occur, such as lymphoma; however, these cases are rare.

What are the signs of adrenal cortical tumors?

Both adrenal adenomas and adrenal cortical cancers can produce excess steroid hormones. Symptoms vary depending on the steroid that is produced. If too much aldosterone, which is a type of steroid hormone, is produced, Conn's syndrome (also known as primary hyperaldosteronism) can develop. Conn’s syndrome most commonly occurs with pituitary adenomas, but it can also occur in the setting of adrenal hyperplasia (an overgrowth of normal adrenal cortical tissue) and adrenal cortical cancers. Signs of Conn's syndrome include elevated blood pressure, decreased levels of potassium in the blood, and decreased levels of a chemical produced by the kidneys called renin in the blood. In most cases of Conn’s syndrome, elevations in blood pressure are mild to moderate. Other symptoms include weakness, muscle cramps, increased thirst, and increased frequency of urination.

Cortisol is a separate steroid hormone produced within the adrenal cortex. If a tumor produces excess cortisol, Cushing's syndrome (also known as hypercortisolism) can develop. This syndrome is seen not only with adrenal tumors, but can also be the result of excessive levels of adrenal cortical stimulating hormone (also known as ACTH, a hormone that is responsible for stimulating the adrenal glands to produce cortisol) produced by the pituitary gland or another tumor in the body. Cushing’s syndrome may also develop in patients who are taking steroids as medication for other disorders. The symptoms of Cushing's syndrome can vary greatly from patient to patient and involve a number of different parts of the body. Symptoms include weight gain and water retention resulting in a round face and collection of fat on the back of the shoulders and neck (so-called “buffalo hump”). Red streaks can appear on the skin known as striae. Excessive hair growth (called hirsutism) can also be seen. Excessive cortisol levels can interfere with the body's immune system predisposing a patient to unusual infections. Patients with Cushing's syndrome are at high risk for development of diabetes. Patients may also have mental changes, including mood swings, irritability, and in the worst case, psychotic episodes. In children, excessive cortisol can lead to premature sexual development and maturation (also called precocious puberty).

Adrenal tumors may also cause excess production of sexual hormones. If excess testosterone is produced, virilization can occur in either men or women. Virilization causes increased masculine characteristics, resulting in deepened voice, loss of hair, and increase in the size of the clitoris in women. Feminization may occur in men with excess estrogen production, and may cause sexual impotence and/ or breast growth (gynecomastia).

Adrenal tumors may also cause symptoms by occupying space in the abdomen. Patients with large adrenal tumors may experience feelings of abdominal fullness or localized pain. Patients may feel as though they are quickly full when eating and may experience weight loss. In some cases of large adrenal tumors, patients may actually feel a mass in their abdomen.

Adrenal cortical cancers stages.

In addition to diagnosing adrenal cortical cancers, the radiographic imaging performed also helps to determine the stage of the patient. In general, patients with adrenal cortical cancer are divided into one of four stages.

Stage I: The cancer is smaller than 5 cm and has not spread outside of the adrenal gland.

Stage II: The cancer is larger than 5 cm and has not spread outside of the adrenal gland.

Stage III: The cancer has spread into the fat surrounding the adrenal gland or has spread to lymph nodes or other organs near the adrenal gland.

Stage IV: The cancer has spread to other parts of the body.

These stages may vary slightly based on the system that a patient’s physician chooses to use; the staging system described above is the most recent way of staging designed by the French Association of Endocrine Surgeons Study Group.

Acortical cancers treatments?

Surgery treatment for Adrenal Cancer

Currently, the only known way to cure adrenal cortical cancers is complete surgical removal of the tumor. Unfortunately, this is only possible for some patients with this disease. At least half of patients with adrenal cortical cancers have metastases or cancer invading into other organs, so that complete removal of the tumor is not possible. The best results with surgical resection have been with an en bloc resection, meaning that the entire tumor is removed in one piece. This also includes removing the entire kidney on the same side as the adrenal cancer. Because of this, it is unusual for adrenal cancers to be removed using a laparoscopic procedure, although as techniques of laparoscopic resection improve, more patients are being treated with this method. Occasionally, adrenal cancers will grow into the large blood vessel that carries blood back from the lower body to the heart (the vena cava). Even in these cases, complete removal of the cancer can sometimes be performed, but will require input from a general surgeon, an urologist, and a vascular surgeon.

Even in cases where the tumor cannot be removed in its entirety, surgical removal of as much tumor as possible can improve symptoms, particularly if they are due to excessive steroid secretion.

Chemotherapy treatmetn for Adrenal Cancer

Chemotherapy refers to a group of medications that are given intravenously or orally as a pill. These drugs travel throughout the body to kill cancer cells. This is one of the big advantages of chemotherapy. If cancer cells have broken off from the tumor and are somewhere else inside the body, chemotherapy has the chance of killing them. A number of different chemotherapeutic agents exist, each with its own side effects. You should discuss the potential side effects of any chemotherapy you may receive with your medical oncologist.

The most common chemotherapy used in treatment of adrenal cortical cancers is mitotane. Mitotane acts to block the hormones produced by the cancer and can also kill adrenal cancer cells. Mitotane or other chemotherapy is almost always used when surgery is not possible, or if surgery is done, but some tumor remains in the body. In many cases, Mitotane is also used for patients after surgery even if every visible cancer cell has been removed. Streptozosin is a second chemotherapy drug that has been shown to work in combination with mitotane. Mitotane with or without streptozosin has been shown in clinical trial to reduce the risk of adrenal cortical cancers growing back after they are removed surgically. Even if cancers cannot be removed surgically because they have spread to other parts of the body, mitotane may cause tumors to shrink and reduce symptoms.

There are a number of other types of chemotherapy used for adrenal cancers. Exactly which chemotherapeutic agents are given varies according to the physician giving them. Based on your own health status and the risks of side effects that you are willing to accept, the choice of chemotherapy can vary.

Radiation Therapy for Adrenal Cancer

Radiation therapy is used in a number of cancers as both the main method of killing cancer cells or in combination with surgery (either before or after). The radiation comes in the form of high-energy x-rays that are delivered to the patient only in the areas at highest risk for cancer. These x-rays are similar to those used for diagnostic x-rays, only of a much higher energy. The high-energy of x-rays in radiation therapy results in damage to the DNA of cells, causing tumor cells to die.

Radiation therapy is not part of the routine management of adrenal cancers, particularly in cases where the cancer is completely removed by surgery. Radiation has been tried in cases where surgical removal of the cancer is incomplete or in cases where the cancer comes back after surgery. In these cases, the radiation is usually delivered daily, Monday through Friday, 5 days a week, for a total of 5 to 7 weeks. In general, the side effects associated with this treatment include fatigue, skin redness and irritation, nausea, and diarrhea.

Other Drug Treatments for Adrenal Cancer

Patients who are treated for adrenal cortical cancers may have symptoms that are due to levels of hormones that are either too high or too low. Physicians may recommend other medications, such as ketoconazole or metyrapone, to treat these symptoms.

Impacts of other cancer on adrenal gland ?

In most cases, when other cancers spread to the adrenal glands, they are treated with chemotherapy that is known to be effective against the original cancer type. In some cases, if the adrenal gland is the only site in the body where the cancer appears to have spread, surgical resection of metastatic cancer and the adrenal gland can be performed, followed by treatment to the primary site where the cancer started. This has been shown to be curative in a small number of patients, particularly in the case of lung cancer.

Related Posts Widget