Pancreatic Neuroendocrine Neoplasms (pNENs)

National Organization for Rare Disorders, Inc.

Skip to the navigation

It is possible that the main title of the report Pancreatic Neuroendocrine Neoplasms (pNENs) is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

The pancreas is a gland located between the stomach, spleen, duodenum and colon transversum. It contains specialized exocrine cells that secrete enzymes that travel to the intestines and aid in digestion as well as endocrine cells, so called islet cells. Pancreatic neuroendocrine neoplasms (pTENs) 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 (e.g. a-cells, ß-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 (e.g. glucose-metabolism). 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. There are several different subtypes of functioning pNENs distinguished by the specific type of hormone that they secrete. Insulinomas and gastrinomas are the most common types of pNENs.

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. 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.

Supporting Organizations

American Cancer Society, Inc.

250 Williams NW St
Ste 6000
Atlanta, GA 30303
Tel: (404)320-3333
Tel: (800)227-2345


3 St. Andrews Place
London, NW1 4LB
United Kingdom
Tel: 2074860341
Fax: 2072242012


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

Carcinoid Cancer Foundation

333 Mamaroneck Avenue #492
White Plains, NY 10605
Tel: (914)683-1001
Fax: (914)683-0183
Tel: (888)722-3132

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

NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Office of Communications & Public Liaison
Bldg 31, Rm 9A06
Bethesda, MD 20892-2560
Tel: (301)496-3583

National Cancer Institute

6116 Executive Blvd Suite 300
Bethesda, MD 20892-8322
Tel: (301)435-3848
Tel: (800)422-6237

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

Neuroendocrine Tumor Research Foundation

20 Park Plaza
Suite 478
Boston, MA 02116
Tel: (617)948-2514
Fax: (617)948-2501

Pancreatic Cancer Action Network

1500 Rosecrans Avenue
Suite 200
Manhattan Beach, CA 90266
Tel: (310)725-0025
Fax: (310)725-0029
Tel: (877)272-6226

Rare Cancer Alliance

1649 North Pacana Way
Green Valley, AZ 85614

For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to 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.

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.

Last Updated:  1/28/2014
Copyright  2014 National Organization for Rare Disorders, Inc.