Pancreatic Neuroendocrine Neoplasms (pNENs)

National Organization for Rare Disorders, Inc.

Important
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

  • islet cell tumors
  • pancreatic endocrine tumors
  • pancreatic islet cell tumors
  • pancreatic neuroendocrine tumors
  • pNET
  • islet cell carcinoma

Disorder Subdivisions

  • ACTHoma
  • calcitoninoma
  • gastrinoma
  • glucagonoma
  • GRFoma
  • insulinoma
  • PPHrPoma
  • Ppoma
  • somatostatinoma
  • VIPoma

General Discussion

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

American Cancer Society, Inc.
250 Williams NW St
Ste 6000
Atlanta, GA 30303
USA
Tel: (404)320-3333
Tel: (800)227-2345
TDD: (866)228-4327
Internet: http://www.cancer.org

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
Tel: (301)496-3583
Email: NDDIC@info.niddk.nih.gov
Internet: http://www2.niddk.nih.gov/

National Cancer Institute
6116 Executive Blvd Suite 300
Bethesda, MD 20892-8322
USA
Tel: (301)435-3848
Tel: (800)422-6237
TDD: (800)332-8615
Email: cancergovstaff@mail.nih.gov
Internet: http://www.cancer.gov

National Coalition for Cancer Survivorship
1010 Wayne Avenue
7th Floor
Silver Spring, MD 20910
Tel: (301)650-9127
Fax: (301)565-9670
Tel: (888)650-9127
Email: info@canceradvocacy.org
Internet: http://www.canceradvocacy.org/

Carcinoid Cancer Foundation
333 Mamaroneck Avenue #492
White Plains, NY 10605
USA
Tel: (914)683-1001
Fax: (914)683-0183
Tel: (888)722-3132
Email: carcinoid@optonline.net
Internet: http://www.carcinoid.org

Pancreatic Cancer Action Network
1500 Rosecrans Avenue
Suite 200
Manhattan Beach, CA 90266
USA
Tel: (310)725-0025
Fax: (310)725-0029
Tel: (877)272-6226
Email: info@pancan.org
Internet: http://www.pancan.org

Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
USA
Internet: http://www.rare-cancer.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

Caring for Carcinoid Foundation
198 Tremont Street
P.O. Box 456
Boston, MA 02116
Tel: (617)948-2514
Fax: (617)948-2501
Email: info@caringforcarcinoid.org
Internet: http://www.caringforcarcinoid.org

Cancer.Net
American Society of Clinical Oncology
2318 Mill Road Suite 800
Alexandria, VA 22314
Tel: (571)483-1780
Fax: (571)366-9537
Tel: (888)651-3038
Email: contactus@cancer.net
Internet: http://www.cancer.net/

CORE
3 St. Andrews Place
London, NW1 4LB
United Kingdom
Tel: 02074860341
Fax: 02072242012
Email: info@corecharity.org.uk
Internet: http://www.corecharity.org.uk

Hageman Foundation
1027 Hampshire Drive
Maryville, TN 37801
Tel: (865)238-5842
Fax: (865)238-5844
Tel: (866)612-8579
Email: Info@HagemanFoundation.org
Internet: http://www.HagemanFoundation.org

For a Complete Report

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".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

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

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

Last Updated:  6/9/2011
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