Pancreatic Neuroendocrine Neoplasms (pNENs)
National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Pancreatic Neuroendocrine Neoplasms (pNENs) is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms Back to top
- islet cell tumors
- pancreatic endocrine tumors
- pancreatic islet cell tumors
- pancreatic neuroendocrine tumors
- islet cell carcinoma
Disorder Subdivisions Back to top
General Discussion Back to top
Pancreatic neuroendocrine neoplasms (pNENs) are an increasingly common group of malignancies that arise within the endocrine tissue of the pancreas. Endocrine tissue is specialized tissue that contains hormone-secreting cells. These cells secrete several different hormones into the blood (endocrine) or to local cells (paracrine, autocrine). These hormones have a variety of functions within the body. Neoplasms that arise from endocrine tissue may also secrete hormones, resulting in excessive levels of these hormones in the body and potentially a wide variety of symptoms. Although there is no difference in diagnosis and therapy, pNENs can be differentiated as functioning or nonfunctioning. Functioning pNENs secrete hormones into the bloodstream, which cause special symptoms; nonfunctioning neoplasms may produce hormones, but no systemic symptoms. Nonfunctioning neuroendocrine neoplasms can still cause symptoms relating to tumor size and location such as obstruction or internal bleeding. There are several different subtypes of functioning pNENs distinguished by the specific type of hormone that they secrete. Most pNENs occur sporadically. They have some different differentiation (G1-3), but all of them have the potential for malignant transformation. Most pNENs occur sporadically, but in some cases, pNENs may occur as part of a larger genetic syndrome such as multiple endocrine neoplasia type 1 (MEN1) or von Hippel Lindau (VHL) syndrome.
Pancreatic cancer as a general term usually refers to pancreatic adenocarcinoma, an aggressive malignant cancer with a poor prognosis. Approximately 95 percent of pancreatic malignancies are adenocarcinomas, for which the prognoses are in general worse than the prognosis of G1- and G2 pNENs. G3 neuroendocrine carcinomas have the same poor prognosis as pancreatic adenocarinomas.
Resources Back to top
American Cancer Society, Inc.
1599 Clifton Road NE
Atlanta, GA 30329
NIH/National Institute of Diabetes, Digestive & Kidney Diseases
Office of Communications & Public Liaison
Bldg 31, Rm 9A06
31 Center Drive, MSC 2560
Bethesda, MD 20892-2560
National Cancer Institute
6116 Executive Blvd
Bethesda, MD 20892-8322
National Coalition for Cancer Survivorship
1010 Wayne Avenue
Silver Spring, MD 20910
Carcinoid Cancer Foundation
333 Mamaroneck Avenue #492
White Plains, NY 10605
Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Caring for Carcinoid Foundation
198 Tremont Street
P.O. Box 456
Boston, MA 02116
American Society of Clinical Oncology
2318 Mill Road
Alexandria, VA 22314
3 St. Andrews Place
London, NW1 4LB
Tel: 020 7486 0341
Fax: 020 7224 2012
For a Complete Report Back to top
This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".
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It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report
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Last Updated: 6/9/2011
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