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    Pancreatic Neuroendocrine Neoplasms (pNENs)

    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

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

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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