Health Information

 

 

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 MyD-H, the Dartmouth-Hitchcock patient portal. You must be a registered MyD-H user for the Lebanon, Manchester, or Nashua locations to access this site.

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

Copyright  1990, 2000, 2011 National Organization for Rare Disorders, Inc.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use. How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.