Endocrine Tumor

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What is Endocrine Tumor ?

A tumor begins when normal cells in the body become abnormal 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, meaning it can spread to and damage the body's healthy tissues if it is not found early and treated.

About the endocrine system

The endocrine system is made up of cells that make hormones. Hormones are chemical substances that are made in the body and carried in the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body. For example, a component of the pancreas is made up of specialized cells clustered together in islands within the organ, called islets of Langerhans. These cells make multiple hormones—the most important being insulin, which is a substance that helps control the amount of sugar in the blood.

Part of the endocrine system is the 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, 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.

About endocrine tumors

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

There are several types of endocrine tumors. For more specific information on each type, select a name below:

Adrenal gland tumor (includes information on adenomas and adrenocortical carcinoma)

Carcinoid tumors (includes information on both lung and gastrointestinal carcinoid tumors)

Islet cell tumor (includes information on gastrinoma, insulinoma, glucagonoma, VIPoma, somatostatinoma, and nonfunctioning tumors)

Neuroendocrine tumor (includes information on Merkel cell cancer, pheochromocytoma, and neuroendocrine carcinoma)

Parathyroid tumor

Pituitary gland tumor

Thyroid cancer

Symptoms and Signs

People with an endocrine tumor may experience the following symptoms or signs. Sometimes, people with an endocrine 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.

Most symptoms of an endocrine tumor are specific to the type of tumor. For more information, see each individual tumor type. The following are some common symptoms of an endocrine tumor:

Thickening or lump in any part of the body

Persistent pain in a specific (localized) area

Anxiety (including panic attacks)

Irritability

Depression

Confusion

Nervousness

Fatigue

Fever

Sweating

Headaches

Nausea

Vomiting

Diarrhea

Changes in bowel or bladder habits

Unexplained weight gain or loss

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

Facial flushing (redness and warm feeling over the face)

Intestinal bleeding

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 an endocrine tumor is diagnosed, relieving symptoms and side effects remains an important part of your 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 can influence the development of a tumor, most do not directly cause the disease. 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 following factors can raise a person's risk of developing specific types of endocrine tumors:

Age. Pheochromocytoma is most common between age 40 and 60. Merkel cell cancer is most common in people older than 70. Thyroid cancer can occur at any age. Anaplastic thyroid cancer is usually diagnosed after age 60. Older infants (10 months and older) and adolescents can develop medullary thyroid cancer, especially if they carry the RET proto-oncogene mutation (see below). For a gastrointestinal carcinoid tumor, the average age at diagnosis is 55 to 65. For a carcinoid tumor of the appendix, the average age at diagnosis is about 40. For a lung carcinoid tumor, the average age at diagnosis is between 45 and 55. Children rarely develop a carcinoid tumor.

Gender. Men are more likely to develop pheochromocytoma than women by a three to two ratio. Men are also more likely to develop Merkel cell cancer than women. Women are two to three times more likely to develop thyroid cancer than men.

Race. A gastrointestinal carcinoid tumor is more common among black people than white people, and black men have a higher risk than black women. Among white people, men and women have the same risk. White people are most likely to develop Merkel cell cancer; however, some black people and Polynesians have developed this type of cancer. White people and Asian people are more likely to develop thyroid cancer.

Family history of multiple endocrine neoplasia, type 1. 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. It is estimated that approximately 10% of gastrointestinal carcinoid tumors are associated with MEN1.

Other family history/genetics. Ten percent of pheochromocytomas are linked to hereditary causes. A history of parathyroid tumors in a family with MEN1 may raise a person's risk of developing a parathyroid tumor. People with certain other hereditary conditions are at higher risk of developing adrenal gland tumors, including Li-Fraumeni syndrome and Carney Complex. Carney Complex can also raise the risk of a pituitary gland tumor. Meanwhile, some types of thyroid cancer are associated with genetics:

An abnormal RET gene, which can be passed from parent to child, may cause medullary thyroid cancer. Not everyone with an altered RET gene will develop cancer. Blood tests and genetic tests can find the gene. Once the altered RET gene is found, a doctor may recommend surgery to remove the thyroid before cancer develops. People with medullary thyroid carcinoma should talk with their doctor about genetic testing to find out if they have a mutation of the RET proto-oncogene. If so, genetic testing of siblings and children will be recommended.

A family history of medullary thyroid cancer increases a person's risk. People with the syndrome multiple endocrine neoplasia, type 2 (MEN2) are also at risk for developing medullary thyroid cancer and other cancers.

A family history of goiters increases the risk of papillary thyroid cancer.

A family history of precancerous polyps in the colon increases the risk of papillary thyroid cancer.

Immune suppression. People with the human immunodeficiency virus (HIV), the virus that causes AIDS, and people whose immune systems are suppressed 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 developing the cancer. 

Radiation exposure. Exposure to moderate levels of radiation therapy may increase the risk of papillary thyroid cancer and follicular thyroid cancer. Such sources of exposure include the following:

Low-dose to moderate-dose x-ray treatments used before 1950 to treat children with acne, tonsillitis, and other head and neck problems may increase the risk of papillary and follicular thyroid cancer.

People who have been treated with radiation therapy for Hodgkin lymphoma or other forms of lymphoma in the head and neck are at an increased risk for developing papillary and follicular thyroid cancer.

One or more exposures to radioactive iodine (also called I-131), especially in childhood, may increase the risk of papillary and follicular thyroid cancer. Sources of I-131 include radioactive fallout from atomic weapons testing during the 1950s and 1960s, nuclear power plant accidents (for example, the 1986 Chernobyl nuclear power plant accident), and environmental releases from atomic weapon production plants.

Diet low in iodine. Iodine is needed for normal thyroid functioning. In the United States, iodine is added to salt to help prevent thyroid problems.

Stomach conditions. People with diseases that damage the stomach and reduce acid production have a greater risk of developing a stomach carcinoid tumor. In particular, this includes pernicious anemia, a type of anemia characterized by very large, abnormal red blood cells.

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it is cancerous and, if so, 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 cancerous tumor 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 endocrine 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 examined by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). There are many types of biopsies. The type of biopsy performed will depend on the location of the tumor. Two of the more common types of biopsies used in the diagnosis of an endocrine tumor include fine needle aspiration and surgical biopsy.

Fine needle aspiration is usually performed in a doctor's office or clinic. A local anesthetic may be injected into the skin near the tumor beforehand to numb the area. The doctor inserts a thin needle into the tumor and removes cells and some fluid. The procedure may be repeated two or three times to get samples from different parts of the tumor. The report done by the pathologist can be positive (meaning there are cancerous cells), negative (meaning there are no cancerous cells), or undetermined (unclear).

If the needle aspiration biopsy is undetermined, the doctor may suggest a biopsy in which the tumor and possibly other tissue in the area will be removed by surgery. This procedure is most often done under general anesthesia and on an outpatient basis.

Laboratory tests. The doctor may need samples of the patient's blood, urine, and stool to check for abnormal levels of hormones, glucose levels, and other substances.

Neurological examination. An evaluation of the central nervous system may include testing of the reflexes, motor and sensory skills, balance and coordination, and mental status.

Imaging tests

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient's vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

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 tumors. Sometimes, a contrast medium (a special dye) is injected into a patient's vein to provide better detail.

Endoscopy. This test allows the doctor to see inside the body with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth, down the esophagus, and into the stomach and small bowel. A colonoscope is a type of endoscope that is inserted through the anus into the colon. It can be used to diagnose a tumor in the lower section of the digestive system.

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

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.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient's body. This substance is absorbed mainly by organs and tissues that use the most energy. Because a tumor tends to use energy actively, it absorbs more of the radioactive substance. However, because some endocrine tumors grow very slowly, it means they use less energy; this means a PET scan may not be as helpful as other tests in the diagnosis.

Radionuclide scanning (OctreoScan). A small amount of a radioactive hormone-like substance is injected into a vein. A special camera is then used to show where the radioactivity accumulates. This procedure is useful for finding where an endocrine tumor has spread, especially if it has spread to the liver. The test takes place over more than one day.

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

Stages

Staging is a way of describing a cancerous tumor, such as where it 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 cancer.

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 if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and 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 (spread) 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.

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

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

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

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

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