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adrenal-cancer

Adrenal Gland Tumor

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What is Adrenal Gland Tumor?

Each person has two adrenal glands—one located on top of each of the body’s two kidneys. These glands are important to the body’s endocrine (hormonal) system. Each adrenal gland has two main parts that function separately:

Adrenal cortex. The outer part of the adrenal gland is called the cortex. The adrenal cortex makes three main hormones: cortisol, aldosterone, and dehydroepiandrosterone (DHEA). These hormones carefully control metabolism and body characteristics, such as hair growth and body shape.

Adrenal medulla. The gland’s inner part is called the medulla. The adrenal medulla makes other hormones: epinephrine, norepinephrine, and dopamine. These hormones control the body’s responses to stress, including the “fight or flight” adrenaline surge

Types of adrenal gland tumors

A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

An adrenal gland tumor can sometimes overproduce hormones. When it does, the tumor is called a functioning tumor. An adrenal gland tumor that does not produce hormones is called a nonfunctioning tumor. A tumor can start in an adrenal gland (called a primary adrenal tumor), or it can begin in another organ, such as the lungs, and then metastasize (spread) to the adrenal glands. The symptoms and treatment of an adrenal gland tumor depend on whether the tumor is functioning or nonfunctioning, what hormone(s) is overproduced, and whether the tumor is a primary adrenal gland tumor or a metastases from cancer of another organ.

This section focuses on primary adrenal gland tumors, which include the following:

Adenoma. Adenoma is the most common type of adrenal gland tumor, making up the majority of all adrenal gland tumors. It is a noncancerous, nonfunctioning tumor of the adrenal cortex. Also called an adrenocortical adenoma, this tumor usually does not cause symptoms and, if it is small, often does not need treatment.

Adrenocortical carcinoma. Although rare, the most common type of cancerous adrenal gland tumors begins in the cortex and is called adrenocortical carcinoma, or adrenal cortical carcinoma. Approximately four to 12 out of one million people develop this type of tumor. Adrenocortical carcinoma can be a functioning or nonfunctioning tumor; if the tumor is functioning, it may produce more than one hormone.

Neuroblastoma. This is a type of childhood cancer that can begin in the adrenal medulla. 

Pheochromocytoma. This type of cancerous neuroendocrine tumor most often begins in the adrenal medulla.

Symptoms & Sign

People with an adrenal gland tumor may experience the following symptoms or signs. Sometimes, people with an adrenal gland tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not a tumor. If you are concerned about a symptom or sign on this list, please talk with your doctor: High blood pressure, Low potassium level, Heart palpitations, Nervousness, Anxiety (panic attacks), Headache, Excessive perspiration, Diabetes, Abdominal pain, Unexplained weight gain or weight loss, Weakness, Abdominal stretch marks, Excessive hair growth, Unusual acne, Change in libido (sex drive)

In addition, pheochromocytoma may cause dangerous surges of the adrenal gland hormones that regulate blood pressure and response to stress. A hormonal surge can cause blood pressure to increase very quickly, increasing the risk of heart attack, stroke, hemorrhage, or sudden death.

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If a tumor is diagnosed, relieving symptoms and side effects remains an important part of medical care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms. 

Risk Factors

A risk factor is anything that increases a person’s chance of developing a tumor. Although risk factors often influence the development of a tumor, most do not directly cause the tumor. Some people with several risk factors never develop a tumor, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The cause of most cancerous adrenal cortical carcinomas is not known. However, people with certain hereditary conditions—such as Li-Fraumeni syndrome, Von Hippel-Lindau syndrome, and Carney Complex—have a higher risk of developing an adrenal gland tumor. People who have a high risk of developing an adrenal gland tumor because of family history should be examined and evaluated yearly by their doctor.

Diagnosis

Doctors use many tests to diagnose a tumor, find out if it is cancerous, and if so, whether it has metastasized. Some tests may also determine which treatments may be the most effective. To diagnose an adrenal gland tumor, blood and urine tests (see below) are done to look for certain substances that may indicate that cancer is present. If there is no evidence that the cancer has spread to the adrenal gland from another part of the body, the diagnosis can be made with a computed tomography (CT or CAT) scan or a magnetic resonance imaging (MRI) scan (see below). Imaging tests may also be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test: Age and medical condition, Type of tumor suspected, Severity of symptoms, Previous test results

In addition to a physical examination, the following tests may be used to diagnose an adrenal gland tumor:

Blood and urine tests. Blood tests can measure the amounts of natural hormones, such as catecholamines and metanephrines, produced during stress, which can detect a functional tumor. A patient may be asked to take a pill on the evening before the blood and urine tests, which helps detect the normal suppression of production of the hormone cortisol. A 24-hour urine sample, which requires the collection of all urine during that timeframe for laboratory testing, may also be needed. This helps the doctor track how quickly various hormones are produced. Tell your doctor about any medications that you take, even over-the-counter drugs, because this information is needed to correctly interpret the results.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. For an adrenal tumor, a narrow, hollow needle is used to collect the tissue; this is called a fine needle biopsy or fine needle aspiration. If the doctor suspects adrenal cancer, a biopsy of the adrenal gland is not recommended because it could help the cancer to spread. However, if the doctor suspects that cancer has spread to the adrenal gland from another area of the body where the cancer started, a biopsy may be done to determine the type of cancer, which can help the doctor plan treatment. The biopsy is performed by a radiologist who uses specialized imaging procedures, such as CT or MRI scans (see below) to direct the needle. The sample removed during the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

CT scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

MRI. An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium is injected into a patient’s vein to provide better detail.

Metaiodobenzylguanidine (MIBG) scan. MIBG is a chemical similar to adrenaline that will collect in a neuroendocrine tumor. A MIBG scan can show a tumor of the adrenal medulla that may not appear in an x-ray. The scan takes place over two consecutive days. On the first day, an injection of MIBG is given in the arm. Several hours later, pictures are taken with a special camera that can detect if or where in the body the MIBG has collected. The following morning, more pictures are taken, and the process may be repeated if needed.

After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is a tumor, these results also help the doctor describe it; this is called staging. 

Stages

Staging is a way of describing where the tumor is located, if it is cancerous, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the tumor’s stage, so staging may not be complete until all of the tests, or even the surgical removal of the tumor or adrenal gland, are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer. This section covers the standard staging system for adrenocortical carcinoma.

One tool that doctors use to describe the stage is the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and whether the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are four stages for adrenocortical carcinoma: stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

How large is the primary tumor, and where is it located? (Tumor, T)

Has the tumor spread to the lymph nodes? (Node, N)

Has the cancer metastasized to other parts of the body? (Metastasis, M)

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no primary tumor.

T1: The tumor is 5 centimeters (cm) or less and has not grown outside of the adrenal gland.

T2: The tumor is larger than 5 cm and has not grown outside of the adrenal gland.

T3: The tumor is any size, and it has grown into the area around the adrenal gland but not to nearby organs.

T4: The tumor is any size and has grown into nearby organs, such as the kidney; diaphragm (the
thin muscle under the lungs and heart that separates the chest from the abdomen); larger blood vessels, called the aorta and the vena cava; pancreas; spleen; and liver. 

Node. The “N” in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near where the tumor started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

NX: The regional lymph nodes cannot be evaluated.

N0 (N plus zero): The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to regional lymph nodes. 

Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.

M0 (M plus zero): The cancer has not spread to other parts of the body.

M1: The cancer has spread to other parts of the body beyond the nearby organs.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage I: The tumor is 5 cm or less and has not grown beyond the adrenal gland or spread to regional lymph nodes or other parts of the body (T1, N0, M0). 

Stage II: The tumor is larger than 5 cm. It has not grown beyond the adrenal gland or spread to regional lymph nodes or other parts of the body (T2, N0, M0).

Stage III: The tumor is described by the following:

It is 5 cm or smaller and has spread to the regional lymph nodes but not to other parts of the body (T1, N1, M0).

It is larger than 5 cm and has spread to the regional lymph nodes but not to other parts of the body (T2, N1, M0).

It is any size and has grown beyond the adrenal gland but not to nearby organs (T3, N0, M0).    

Stage IV: The tumor is described by the following:

It is any size and has grown into the area around the adrenal gland but not to nearby organs. The tumor has spread to regional lymph nodes but not to other parts of the body (T3, N1, M0).

It is any size and has spread to nearby organs but not to the lymph nodes or other parts of the body beyond the nearby organs (T4, N0, M0).

It is any size and has spread to nearby organs. The tumor has spread to the regional lymph nodes but not to other parts of the body beyond the nearby organs (T4, N1, M0).

The tumor has spread to other parts of the body (any T, any N, M1).

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

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