Skip to content

    Pancreatic Cancer Health Center

    Font Size

    Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Pancreatic Neuroendocrine Tumors (Islet Cell Tumors)

    Incidence and Mortality

    They are uncommon cancers with about 1,000 new cases per year in the United States.[1] They account for 3% to 5% of pancreatic malignancies and overall have a better prognosis than the more common pancreatic exocrine tumors.[1,2] Five-year survival is about 55% when the tumors are localized and resected but only about 15% when the tumors are not resectable.[2] Overall 5-year survival rate is about 42%.[1]

    Figure 1. Cancer of the Pancreas: Relative Survival Rates (%) by Histologic Subtype, Ages 20+, 12 SEER Areas, 1988-2001. Key, C. Ch 7: Cancer of the pancreas. In: Ries LAG, Young JL, Keel GE, et al. (eds). SEER Survival Monograph: Cancer Survival Among Adults: U.S. SEER Program, 1988-2001, Patient and Tumor Characteristics. National Cancer Institute, SEER Program, NIH Pub. No. 07-6215, Bethesda, MD, 2007.


    Tumors of the endocrine pancreas are a collection of tumor cell types collectively referred to as pancreatic neuroendocrine tumors (NETs). These tumors originate in islet cells. Although they may be similar or identical in histologic appearance to carcinoid tumors of the gastrointestinal tract, differences in their underlying biology and likely differences in response to therapeutic agents suggest that they should be treated and investigated as a distinct entity.[3]

    Most pancreatic NETs are sporadic, but some occur as part of the autosomal dominant multiple endocrine neoplasia type-1 (MEN-1) inherited syndrome consisting of tumors of the anterior pituitary, parathyroid, and endocrine pancreas glands, which results from the inactivation of the tumor suppressor gene Menin located on chromosome 11q13.[4] When part of the MEN-1 syndrome, there may be multiple pancreatic tumors.

    Islet tumors may either be functional (produce one or more active hormones) or nonfunctional.[4] The functional tumors, which usually present with symptoms of hormone hypersecretion, include:

    • Gastrinoma.
    • Insulinoma.
    • Glucagonoma.
    • Somatostatinoma.
    • VIPoma.

    Prognostic Factors

    Most islet cell cancers are functional, but about 15% are nonfunctional, with presentations similar to the far more common exocrine adenocarcinomas of the pancreas.[5,6,7] Because of the presence of several cell types in the pancreatic islets (alpha, beta, delta, A, B, C, D, E, F), the term, islet cell tumors, refers to at least five distinct cancers that, when functional, produce unique metabolic and clinical characteristics. The clinical manifestations in functional tumors may result from the distinctive metabolic effects of the polypeptide(s) secreted by the cancer cells rather than from tumor bulk or metastatic disease. Functional tumors may even be too small to be detected by conventional imaging techniques.

    1 | 2 | 3 | 4
    1 | 2 | 3 | 4
    Next Article:

    Today on WebMD

    human pancreas
    Do you know what they are?
    man with a doctor
    Our health check will steer you in the right direction.
    sauteed cherry tomatoes
    Fight cancer one plate at a time.
    Lung cancer xray
    See it in pictures, plus read the facts.
    Integrative Medicine Cancer Quiz
    Patrick Swayzes Widow Healing From Loss
    Pets Improve Your Health
    Resolved To Quit Smoking