Cancer - pancreas; Cancer - pancreatic; Pancreatic cancer; Islet cell tumors; Islet of Langerhans tumor; Neuroendocrine tumors; Peptic ulcer - islet cell tumor; Hypoglycemia - islet cell tumor; Zollinger-Ellison syndrome; Verner-Morrison syndrome; Gastrinoma; Insulinoma; VIPoma; Somatostatinoma; Glucagonoma; Pancreatic islet cell tumor DefinitionPancreatic neuroendocrine tumors (NET) are rare tumors of the pancreas that start from a type of cell called the islet cell. CausesIn the healthy pancreas, cells called islet cells produce hormones that regulate several bodily functions. These include blood sugar level and the production of stomach acid. Tumors that arise from islet cells of the pancreas can also produce a variety of hormones, which can lead to specific symptoms. Pancreatic NETs can be noncancerous (benign) or cancerous (malignant). NETs include:
A family history of multiple endocrine neoplasia, type I (MEN I) is a risk factor for the development of NETs. SymptomsSymptoms depend on which hormone is made by the tumor. For example, insulinomas produce insulin, which lowers blood sugar level. Symptoms may include:
If your blood sugar level gets too low, you may faint, have a seizure, or even go into a coma. Gastrinomas make the hormone gastrin, which tells the body to make stomach acid. Symptoms may include:
Glucagonomas make the hormone glucagon, which helps the body raise blood sugar level. Symptoms can include:
Somatostatinomas make the hormone somatostatin. Symptoms may include:
VIPomas make the hormone vasoactive intestinal peptide (VIP) which is involved in maintaining the balance of sodium, potassium and other minerals in the GI tract. VIPomas may cause:
Exams and TestsYour health care provider will take your medical history and perform a physical exam. Blood tests may vary, depending upon the symptoms, but may include:
Imaging tests may be done: A blood sample may also be taken from a vein in the pancreas for testing. Sometimes, surgery is needed to diagnose and treat this condition. During this procedure, the surgeon examines the pancreas by hand and with ultrasound. TreatmentTreatment depends on the type of tumor and if it is cancerous. Cancerous tumors can grow quickly and spread to other organs. They may not be treatable. Tumors are often removed with surgery, if possible. If cancer cells spread to the liver, a part of the liver may also be removed, if possible. If the cancer is widespread, chemotherapy may be used to try to shrink the tumors. If the abnormal production of hormones is causing symptoms, you may receive medicines to counteract their effects. For example, with gastrinomas, the overproduction of gastrin leads to too much acid in the stomach. Medicines that block stomach acid release can reduce symptoms. Support GroupsYou can ease the stress of illness by joining a cancer support group. Sharing with others who have common experiences and problems can help you not feel alone. Outlook (Prognosis)You may be cured if the tumors are surgically removed before they have spread to other organs. If tumors are cancerous, chemotherapy may be used, but it usually cannot cure people. Life-threatening problems (such as very low blood sugar) can occur due to excess hormone production, or if the cancer spreads throughout the body. Possible ComplicationsComplications of these tumors include:
When to Contact a Medical ProfessionalContact your provider if you develop symptoms of these tumors, especially if you have a family history of MEN I. PreventionThere is no known prevention for these tumors. ReferencesKhreiss MR, Riall TS. Pancreatic neuroendocrine tumors of the pancreas excluding gastrinoma. In: Cameron JL, Cameron AM, eds. Current Surgical Therapy. 14th ed. Philadelphia, PA: Elsevier; 2023:632-638. National Cancer Institute website. Pancreatic neuroendocrine tumors (islet cell tumors) treatment (PDQ) - health professional version. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq. Updated August 19, 2022. Accessed May 6, 2024. National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology (NCCN guidelines). Neuroendocrine and adrenal tumors. Version 1. 2023. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated August 2, 2023. Accessed May 6, 2024. National Comprehensive Cancer Network website. NCCN guidelines for patients. Neuroendocrine tumors. 2022. www.nccn.org/patients/guidelines/content/PDF/neuroendocrine-patient.pdf. Accessed May 6, 2024. | ||
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Review Date: 3/31/2024 Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team. View References The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. No warranty of any kind, either expressed or implied, is made as to the accuracy, reliability, timeliness, or correctness of any translations made by a third-party service of the information provided herein into any other language. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited. | ||
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Endocrine glands
Pancreas
