Pancreatic neuroendocrine tumor

Title

Other Names:

Neuroendocrine tumor of pancreas; Pancreatic NET; PNET; Neuroendocrine tumor of pancreas; Pancreatic NET; PNET; Well-differentiated NEN of pancreas; Well-differentiated neuroendocrine neoplasm of pancreas; Well-differentiated pancreatic NEN; Well-differentiated pancreatic neuroendocrine neoplasm; Pancreatic endocrine tumor; Islet cell tumor; Pancreatic neuroendocrine neoplasm See More

Summary 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]

Last updated: 5/18/2018

Treatment 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.

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Find a Specialist Find a Specialist

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

The Neuroendocrine Tumor Research Foundation has a Doctor Database for help finding a physician specializing in treating neuroendocrine tumors. They also provide a link to a list of multidisciplinary diagnosis and treatment centers on the same web page.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

General Clinical Trials & Research

The Neuroendocrine Tumor Research Foundation is a non-profit organization to support neuroendocrine tumor (NET) research. Their mission is to accelerate scientific discovery that will help create new and more effective therapies for NETs. Each year they seek and fund basic, translational and clinical research proposals with the potential to further understanding of NETs and accelerate the development of diagnostic and therapeutic interventions for patients.

Organizations 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

National Pancreas Foundation
3 Bethesda Metro Center, Suite 700
Bethesda, MD 20814
Toll-free: 866-726-2737
Telephone: 301-961-1508
E-mail: info@pancreasfoundation.org
Website: http://pancreasfoundation.org/
NET Patient Foundation (UK and Ireland)
Holly House (2nd Floor)
74 Upper Holly Walk
Leamington Spa
Warwickshire, CV32 4JL United Kingdom
Toll-free: 0800 434 6476 (Helpline)
Telephone: 01926 883487 (Office)
E-mail: kulli@netpatientfoundation.org
Website: http://www.netpatientfoundation.org/
Neuroendocrine Tumor Research Foundation (NET Research Foundation)
321 Columbus Avenue, Suite 5R
Boston, MA 02116
Telephone: 617-946-1780
E-mail: info@netrf.org
Website: https://netrf.org/
Pancreatic Cancer Action Network
2141 Rosecrans Ave, Suite 7000
El Segundo, CA 90245
Toll-free: 877-272-6226
Telephone: 310-725-0025
Fax: 310-725-0029
E-mail: info@pancan.org
Website: http://www.pancan.org
Pancreatica
Cancer Patients Alliance
312 Fountain Avenue
Pacific Grove, CA 93950
Telephone: (831) 658-0600
E-mail: info@pancreatica.org
Website: http://pancreatica.org/

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Learn More 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.

Related Diseases Related Diseases

The following diseases are related to Pancreatic neuroendocrine tumor. If you have a question about any of these diseases, you can contact GARD.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

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References References

Information on Pancreatic NETs. Neuroendocrine Tumor Research Foundation (NETRF). https://netrf.org/pancreatic-nets/information-on-pancreatic-nets/. Accessed 5/18/2018.
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.
Yu R. Neuroendocrine tumor of pancreas. Orphanet. November 2014; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=97253.

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