Endocrine Tumor

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

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

Stages

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.

Treatment

Treatment options and recommendations depend on several factors, including the type and stage of the tumor, if it is cancerous; possible side effects; and the patient's preferences and overall health. Your doctor may also recommend genetic testing of your tumor to help plan treatment. Learn more about making treatment decisions.

Surgery

The purpose of surgery is typically to remove the entire tumor, along with some of the healthy tissue around it, called the margin. A surgical oncologist is a doctor who specializes in treating cancer using surgery. If the tumor cannot be removed entirely, “debulking” surgery may be performed. Debulking surgery is a procedure in which the goal is to remove as much of the tumor as possible.

Side effects of surgery include weakness, fatigue, and pain for the first few days following the procedure.

Chemotherapy

Chemotherapy is the use of drugs to kill tumor cells, usually by stopping the cells' ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating tumors with medication. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about chemotherapy and preparing for treatment. The medications used to treat tumors are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill tumor cells. A doctor who specializes in giving radiation therapy to treat a tumor is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen usually consists of a specific number of treatments given over a set period of time.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished.

Hormone therapy

The goal of hormone therapy is often to lower the levels of hormones in the body. Hormone therapy may be given to help stop the tumor from growing or to relieve symptoms caused by the tumor. In addition, for thyroid cancer, hormone therapy will be given if the thyroid gland has been removed, to replace the hormone that is needed by the body to function properly.

Immunotherapy

Immunotherapy (also called biologic therapy) is designed to boost the body's natural defenses to fight the tumor. It uses materials made either by the body or in a laboratory to bolster, target, or restore immune system function. Examples of immunotherapy include cancer vaccines, monoclonal antibodies, and interferons.

Alpha interferon is a form of biologic therapy given as an injection under the skin. This is sometimes used to help relieve symptoms caused by the tumor, but it can have severe side effects including fatigue, depression, and flu-like symptoms. Learn more about immunotherapy.

Targeted therapy

Targeted therapy is a treatment that targets the tumor's specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of tumor cells while limiting damage to normal cells, usually leading to fewer side effects than other cancer medications.

Recent studies show that not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them.

Depending on the type of endocrine tumor, targeted therapy may be a possible treatment option. For instance, targeted therapies, such as sunitinib (Sutent) and everolimus (Afinitor), have been approved for treating advanced islet cell tumors. Early results of clinical trials (research studies) with targeted therapy drugs for other types of endocrine tumors are promising, but more research is needed to prove they are effective. Learn more about these treatments in the Current Research section. Because research on targeted therapy is ongoing, please talk with your doctor to learn about targeted therapy treatment options. Learn more about targeted treatments.

Recurrent endocrine tumor

Once your treatment is complete and there is a remission (absence of symptoms; also called “no evidence of disease” or NED), talk with your doctor about the possibility of the tumor returning. Many survivors feel worried or anxious that the tumor will come back.

If the tumor does return after the original treatment, it is called a recurrent tumor. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

People with a recurrent tumor often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with recurrence.

Metastatic endocrine tumor

If a cancerous tumor has spread to another location in the body, it is called metastatic cancer.

Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.