Neuroendocrine Tumor

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What is Neuroendocrine Tumor ?

About the endocrine system and endocrine tumors

The endocrine system consists of cells that make hormones. Hormones are chemical substances that are formed in the body and carried in the bloodstream to have a specific effect on the activity of other organs or cells in the body.

A tumor begins when normal cells change and grow uncontrollably, forming a mass. A tumor can be benign or malignant. A benign tumor is not cancerous and usually can be removed without it causing much harm. A malignant tumor is cancerous; if it is not found early and treated, it may be harmful and may spread to and damage the body’s healthy tissues and organs.

An endocrine tumor is a growth that affects the parts of the body that secrete hormones. Because an endocrine tumor arises from cells that produce hormones, the tumor itself can produce hormones and cause serious illness.

About neuroendocrine tumors

A neuroendocrine tumor begins in the hormone-producing cells of the body’s neuroendocrine system, which is made up of cells that are a cross between traditional endocrine cells (or hormone-producing cells) and nerve cells. Neuroendocrine cells are found throughout the body in organs such as the lungs and gastrointestinal tract (including the stomach and intestines) and perform specific functions, such as regulating the air and blood flow through the lungs and controlling the speed at which food is moved through the gastrointestinal tract.

Types of neuroendocrine tumors

There are many types of neuroendocrine tumors. This section focuses on three specific types: pheochromocytoma, Merkel cell cancer, and neuroendocrine carcinoma. Learn more about other types of cancer that begin in hormone-producing cells, including endocrine tumors,carcinoid tumors, thymoma, thyroid cancer, and islet cell tumors.

Pheochromocytoma. Pheochromocytoma is a cancer of the chromaffin cells (the cells in the body that release the hormone adrenaline during times of stress). Pheochromocytoma most often occurs in the adrenal medulla, the area inside the adrenal glands. Each person has two adrenal glands, one located on top of each kidney. This type of tumor increases the production of the hormones adrenaline and noradrenaline, which increase blood pressure and heart rate. Pheochromocytoma can be life-threatening because the tumor may release large amounts of adrenaline into the bloodstream after injury. Eighty percent (80%) of people with pheochromocytoma have a tumor on only one adrenal gland, 10% have tumors on both glands, and 10% have a tumor outside of the adrenal glands.

Merkel cell cancer. Merkel cell cancer, also called neuroendocrine carcinoma of the skin or trabular cancer, is a highly aggressive, rare cancer. It starts from hormone-producing cells just beneath the skin and in the hair follicles, and it is found in the head and neck region.

Neuroendocrine carcinoma. Approximately 60% of neuroendocrine tumors are not able to be described as a specific type of cancer other than neuroendocrine carcinoma. Neuroendocrine carcinoma can be found in a number of places in the body, including the lungs, brain, and gastrointestinal tract.

Risk Factors : A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, 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 following factors can raise a person's risk of developing a neuroendocrine tumor:

Age. Pheochromocytoma is most common between the ages of 40 and 60. Merkel cell cancer is most common in people older than 70.

Gender. Men are more likely to develop pheochromocytoma than women. For every two women who develop pheochromocytoma, three men will develop the disease. Men are also more likely to develop Merkel cell cancer than women.

Race/ethnicity. White people are most likely to develop Merkel cell cancer; however, some black people and people of Polynesian descent have developed the disease.

Family history. Ten percent of pheochromocytomas are linked to hereditary causes. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing a tumor of three glands: pituitary, parathyroid, and pancreas. Multiple endocrine neoplasia type 2 (MEN2) is a hereditary condition associated with medullary thyroid cancer and other types of cancer, including pheochromocytoma.

Immune system suppression. People with human immunodeficiency virus (HIV), the virus that causes acquired immune deficiency syndrome (AIDS), and people whose immune systems are lowered because of organ transplantation have a higher risk of developing a neuroendocrine tumor.

Arsenic exposure. Exposure to the poison arsenic may increase the risk of Merkel cell cancer.

Sun exposure. Because Merkel cell cancer often occurs on the sun-exposed areas of the head and neck, many doctors think that sun exposure may be a risk factor for this type of cancer.

Symptoms :  People with a neuroendocrine tumor may experience the following symptoms or signs. Sometimes, people with a neuroendocrine tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Pheochromocytoma

Hypertension (high blood pressure)

Anxiety attacks

Fever

Headaches

Sweating

Nausea

Vomiting

Clammy skin

Rapid pulse

Heart palpitations

Merkel cell cancer

Painless, firm, shiny lumps on the skin that can be red, pink, or blue in color

Neuroendocrine carcinoma

Hyperglycemia (a high level of a sugar called glucose in the blood, which causes frequent urination, increased thirst, and increased hunger)

Hypoglycemia (a low level of glucose in the blood, which causes fatigue, nervousness and shakiness, dizziness or light-headedness, sweating, seizures, and fainting)

Diarrhea

Persistent pain in a specific area

Loss of appetite/weight loss

Persistent cough or hoarseness

Thickening or lump in any part of the body

Changes in bowel or bladder habits

Unexplained weight gain or loss

Jaundice (yellowing of the skin and whites of the eyes)

Unusual bleeding or discharge

Persistent fever or night sweats

Headache

Anxiety

Gastric ulcer disease

Skin rash

Some people also experience nutritional deficiencies (such as niacin and protein deficiency) before a diagnosis, while others have this symptom later.

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 cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer 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. 

Diagnosis : Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may 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 cancer suspected

Severity of symptoms

Previous test results

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

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). 

Blood/urine tests. The doctor may need samples of the patient’s blood and urine to check for abnormal levels of hormones and other substances. Urine tests check for increased levels of adrenaline in the body. Large amounts of adrenaline can be a sign of pheochromocytoma. The glucagon stimulation test and clonidine suppression test are blood tests that measure adrenaline levels over a period of time for people who sometimes have symptoms of pheochromocytoma. During the glucagon stimulation test, glucagon (a hormone produced by the pancreas that helps the body process carbohydrates) is injected into a vein, and blood samples are drawn at specific intervals to measure the adrenaline levels. During the clonidine (Catapres) suppression test, a tablet of the drug clonidine is swallowed, and blood samples are taken at regular intervals over the next three hours. Clonidine is used to lower adrenaline levels in the blood, so if these levels do not decrease during testing, it may be a sign of a tumor. Blood pressure and heart rate are carefully monitored during these tests.

X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation.

Computed tomography (CT or CAT) 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 a tumor. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.

Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy (see Treatment).

Stages : Staging is a way of describing where the cancer is located, 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 cancer's stage, so staging may not be complete until all the tests 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 cancers.

There is no standard staging system for a neuroendocrine tumor. Staging for pancreatic neuroendocrine tumors is the same as staging for pancreatic cancer. The following staging system is commonly used for Merkel cell cancer:

Stage IA: The primary tumor is smaller than 2 centimeters (cm) and has not spread to the lymph nodes or other parts of the body. Lymph nodes are tiny, bean-shaped organs that help fight infection.

Stage IB: The primary tumor is equal to or larger than 2 cm and has not spread to the lymph nodes or other parts of the body.

Stage II: The cancer has spread to the regional lymph nodes, but it has not spread to other parts of the body.

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

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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