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Overview

Name: Neuroendocrine (islet cell) tumor

Description: the other kind of "pancreas cancer"

This casebook is published and has been read 369 times.

The author of this casebook has identified the following medical topics as being highly relevant to this casebook.

  • Neuroendocrine Neoplasm -- Tumors whose cells possess secretory granules and originate from the neuroectoderm, i.e., the cells of the ectoblast or epiblast that program the neuroendocrine system. Common properties across most neuroendocrine tumors include ectopic hormone production (often via APUD CELLS), the presence of tumor-associated antigens, and isozyme composition.

  • Neuroendocrine Carcinoma -- A group of carcinomas which share a characteristic morphology, often being composed of clusters and trabecular sheets of round "blue cells", granular chromatin, and an attenuated rim of poorly demarcated cytoplasm. Neuroendocrine tumors include carcinoids, small ("oat") cell carcinomas, medullary carcinoma of the thyroid, Merkel cell tumor, cutaneous neuroendocrine carcinoma, pancreatic islet cell tumors, and pheochromocytoma. Neurosecretory granules are found within the tumor cells. (Segen, Dictionary of Modern Medicine, 1992)

  • Islet Cell Tumor -- An uncommon benign or malignant neoplasm, arising from the islet cells of the pancreas. Based on the clinical manifestations, it is classified as functioning or non-functioning.

Notes

What are islet cell tumors? 

An islet cell tumor is a mass of abnormal cells that forms in the endocrine (hormone -producing) tissues of the pancreas. Islet cell tumors may be benign (noncancer) or malignant (cancerous). Islet cell cancer is rare.

 

The pancreas is about 6 inches long and is shaped like a thin pear, wider at one end and narrower at the other. The pancreas lies behind the stomach, inside a loop formed by part of the small intestine. The broader right end of the pancreas is called the head, the middle section is called the body, and the narrow left end is the tail.

 

The pancreas has two basic jobs in the body. It produces digestive juices that help break down (digest) food, and hormones (such as insulin) that regulate how the body stores and uses food. The area of the pancreas that produces digestive juices is called the exocrine pancreas. About 95% of pancreatic cancers begin in the exocrine pancreas. The hormone-producing area of the pancreas has special cells called islet cells and is called the endocrine pancreas. Only about 5% of pancreatic cancers start here. This summary has information on cancer of the endocrine pancreas (islet cell cancer). (See the PDQ summary on Pancreatic Cancer Treatment for more information on cancer of the exocrine pancreas.)

 

The islet cells in the pancreas make many hormones, including insulin, which help the body store and use sugars. When islet cells in the pancreas become cancerous, they may make too many hormones. Islet cell cancers that make too many hormones are called functioning tumors. Other islet cell cancers may not make extra hormones and are called nonfunctioning tumors. Tumors that do not spread to other parts of the body can also be found in the islet cells. These are called benign tumors and are not cancer. A doctor will need to determine whether the tumor is cancer or a benign tumor.

 

A doctor should be seen if there is pain in the abdomen, diarrhea, stomach pain, a tired feeling all the time, fainting, or weight gain without eating too much.

 

If there are symptoms, the doctor will order blood and urine tests to see whether the amounts of hormones in the body are normal. Other tests, including x-rays and special scans, may also be done.

 

The chance of recovery (prognosis) depends on the type of islet cell cancer the patient has, how far the cancer has spread, and the patient's overall health.

 Stages of islet cell cancer 

Once islet cell cancer is found, more tests will be done to find out if cancer cells have spread to other parts of the body. This is called staging. The staging system for islet cell cancer is still being developed. These tumors are most often divided into one of three groups:

 

  1. islet cell cancers occurring in one site within the pancreas,
  2. islet cell cancers occurring in several sites within the pancreas, or
  3. islet cell cancers that have spread to lymph nodes near the pancreas or to distant sites.

 

Treatment of islet cell cancer depends on the type of tumor, the stage, and the patient’s general health. The following types of islet cell tumors are found:

  Gastrinoma 

The tumor makes large amounts of a hormone called gastrin, which causes too much acid to be made in the stomach. Ulcers may develop as a result of too much stomach acid.

  Insulinoma 

The tumor makes too much of the hormone insulin and causes the body to store sugar instead of burning the sugar for energy. This causes too little sugar in the blood, a condition called hypoglycemia.

  Glucagonoma 

This tumor makes too much of the hormone glucagon and causes too much sugar in the blood, a condition called hyperglycemia.

  Miscellaneous 

Other types of islet cell cancer can affect the pancreas and/or small intestine. Each type of tumor may affect different hormones in the body and cause different symptoms.

  Recurrent 

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the pancreas or in another part of the body.

How islet cell cancer is treated 

Different types of treatment are available for patients with islet cell cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

 

Three types of standard treatment are used:

 

 

Surgery is the most common treatment of islet cell cancer. The doctor may take out the cancer and most or part of the pancreas. Sometimes the stomach is taken out (gastrectomy) because of ulcers. Lymph nodes in the area may also be removed and looked at under a microscope to see if they contain cancer.

 

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in the vein or muscle. Chemotherapy is called a systemic treatment because the drug enters the bloodstream, travels through the body, and can kill cancer cells throughout the body.

 

Hormone therapy uses hormones to stop the cancer cells from growing or to relieve symptoms caused by the tumor.

 

Hepatic arterial occlusion or embolization uses drugs or other agents to reduce or block the flow of blood to the liver in order to kill cancer cells growing in the liver.

  Treatment in a clinical trial 

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

 

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

 

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

 

Clinical trials are taking place in many parts of the country. In the following lists of treatments for the different types of islet cell tumors, a link to search results for current clinical trials is included for each section. These have been retrieved from NCI's clinical trials database. For some types of islet cell tumors, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

 

Bookmarks The following information, which has been distilled by the casebook author from this and other websites is particularly relevant to this casebook.
Bookmarks - Web
Web Page: nformation and LinkNotesConcepts
 MedlinePlus Medical Encyclopedia: Pancreatic islet cell tumor Pancreas Islet Cell Tumor, U.S. National Library of Medicine (Neuroendocrine Neoplasm)
 
 http://search2.google.cit.nih.gov/search?site=NIH_Master&client=NIHNEW_frontend&proxystylesheet=NIHNEW_frontend&output=xml_no_dtd&filter=0&getfields=*&q=neuroendocrine tumor&btnG.x=0&btnG.y=0&btnG=search Clinical trials of new treatments for neuroendocrine tumors (Neuroendocrine Neoplasm)
 
 http://search2.google.cit.nih.gov/search?site=NIH_Master&client=NIHNEW_frontend&proxystylesheet=NIHNEW_frontend&output=xml_no_dtd&filter=0&getfields=*&q=pancreatic neuroendocrine&btnG.x=0&btnG.y=0&btnG=search Clinical trials of new treatments for pancreatic neuroendocrine tumors or PNETs (Neuroendocrine Carcinoma)
 
 Islet Cell Tumors (Pancreatic) Treatment - National Cancer Institute U.S. National Cancer Institute, consumer and professional information in English and en Espanol (Neuroendocrine Neoplasm)
 
 World J Gastroenterol From the professional medical literature. A 2005 review of liver transplantation for treatment of islet cell neuroendocrine tumors (Islet Cell Tumor)
 
 Hepatic surgery for metastases from neuroendocrine...[Surg Oncol Clin N Am. 2003] - PubMed Result From the professional medical literature. A 2003 review of liver surgery for metastases of neuroendocrine tumors (Neuroendocrine Carcinoma)
 
 Management of neuroendocrine liver metastases. [Am J Surg. 2004] - PubMed Result From the professional medical literature. A 2004 review of treatment of liver metastatses (Islet Cell Tumor)
 

This web-site is for informational purposes only and is not intended as a substitute for advice from your doctor. It should not to be used for self-diagnosis or treatment.