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Pancreatic islet cell tumor

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

A pancreatic islet cell tumor is a rare tumor of the pancreas that starts from a type of cell called the islet cell.

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Endocrine glands
Pancreas

Causes

In 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 islet cell tumors can be noncancerous (benign) or cancerous (malignant).

Islet cell tumors include:

A family history of multiple endocrine neoplasia, type I (MEN I) is a risk factor for the development of islet cell tumors.

Symptoms

Symptoms 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 salts, sodium, potassium and other minerals in the GI tract. VIPomas may cause:

Exams and Tests

Your 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.

Treatment

Treatment 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 and 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 Groups

You 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 Complications

Complications of these tumors include:

When to Contact a Medical Professional

Call your provider if you develop symptoms of these tumors, especially if you have a family history of MEN I.

Prevention

There is no known prevention for these tumors.

Related Information

Tumor
Benign
Zollinger-Ellison syndrome
Endocrine glands
Multiple endocrine neoplasia (MEN) I

References

Foster DS, Norton JA. Management of pancreatic islet cell tumors excluding gastrinoma. In: Cameron AM, Cameron JL, eds. Current Surgical Therapy. 13th ed. Philadelphia, PA: Elsevier; 2020:581-584.

National Cancer Institute website. Pancreatic cancer treatment (PDQ) - health professional version. www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq. Updated January 2, 2020. Accessed February 25, 2020.

National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology (NCCN guidelines). Neuroendocrine and adrenal tumors. Version 1.2019. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. Updated March 5, 2019. Accessed February 25, 2020.

National Comprehensive Cancer Network website. NCCN guidelines for patients. Neuroendocrine tumors. 2018. www.nccn.org/patients/guidelines/content/PDF/neuroendocrine-patient.pdf.

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Review Date: 2/6/2020  

Reviewed By: Todd Gersten, MD, Hematology/Oncology, Florida Cancer Specialists & Research Institute, Wellington, FL. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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