Rare Gastroenterology News

Disease Profile

Pancreatic neuroendocrine tumor

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

no.svg

Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

no.svg

X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

no.svg

X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

no.svg

Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

no.svg

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

no.svg

Not applicable

no.svg

Other names (AKA)

Neuroendocrine tumor of pancreas; Pancreatic NET; PNET;

Summary

A pancreatic neuroendocrine tumor, also called an islet cell tumor, is a type of neuroendocrine tumor (NET) that typically arises in the pancreas. However in some cases, a pancreatic NET occurs outside of the pancreas. A NET arises from cells that produce hormones, so the tumor can also produce hormones.[1] It may be benign (not cancerous) or malignant (cancerous). Pancreatic NETs usually grow slowly over many years, but there are fast-growing forms.[1]

Pancreatic NETs are called either functional or nonfunctional. A functional pancreatic NET causes specific symptoms because it makes extra hormones, such as gastrin, insulin, or glucagon.[1][2] Examples of types of functional pancreatic NETs include insulinomas, glucagonomas, gastrinomas, VIPomas, and somatostatinomas.[1] Symptoms depend on the type of hormone being made.[2] A nonfunctional pancreatic NET generally does not cause specific symptoms, but may eventually cause symptoms relating to its location and size as it grows or spreads.[2] Pancreatic NETs can be hard to diagnosis, often not identified until 5 to 10 years after they begin to grow.[1]

Most pancreatic NETs are not inherited and occur sporadically in people with no family history of NETs. However, about 10% are associated with a hereditary cancer or tumor syndrome such as multiple endocrine neoplasia type 1 (MEN1), which has autosomal dominant inheritance.[3]

Treatment options for each person depend on many factors including the type of tumor, its location, whether it has spread to other parts of the body (metastasized), and symptoms present.[1] Treatment options may include surgery to remove all or part of the tumor (and sometimes the lymph nodes), and/or nonsurgical treatments to shrink the tumor, stop it from growing, or alleviate symptoms.[1] The chance of recovery (prognosis) also depends upon these factors and differs from person to person.[2]

Treatment

The resources below provide information about treatment options for this condition. If you have questions about which treatment is right for you, talk to your healthcare professional.

Management Guidelines

  • The North American Neuroendocrine Tumor Society has published several consensus guidelines relating to the medical treatment and management of neuroendocrine tumors. Guidelines are developed pursuant to National Institute of Health (NIH) standards and serve as important references for practicing physicians.

    Organizations

    Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

    Organizations Supporting this Disease

      Learn more

      These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

      Where to Start

      • Cancer.net provides oncologist-approved cancer information from the American Society of Clinical Oncology and has information about Pancreatic neuroendocrine tumor.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • The National Cancer Institute provides the most current information on cancer for patients, health professionals, and the general public.
      • The NET Patient Foundation has an online handbook that aims to serve as a guide to living with neuroendocrine tumors.
      • The Neuroendocrine Tumor Research Foundation provides information about Pancreatic neuroendocrine tumor.
      • The Pancreatic Cancer Action Network provides information about Pancreatic neuroendocrine tumor.

        In-Depth Information

        • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
        • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.

          References

          1. Information on Pancreatic NETs. Neuroendocrine Tumor Research Foundation (NETRF). https://netrf.org/pancreatic-nets/information-on-pancreatic-nets/. Accessed 5/18/2018.
          2. Pancreatic Neuroendocrine Tumors (Islet Cell Tumors) Treatment (PDQ®)–Patient Version. National Cancer Institute. March 22, 2018; https://www.cancer.gov/types/pancreatic/patient/pnet-treatment-pdq.
          3. Yu R. Neuroendocrine tumor of pancreas. Orphanet. November 2014; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=97253.