Learn About Pancreatic Islet Cell Tumor

View Main Condition: Pancreatic Cancer

What is the definition of Pancreatic Islet Cell Tumor?

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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What are the alternative names for 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

What are the causes of Pancreatic Islet Cell Tumor?

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:

  • Gastrinoma (Zollinger-Ellison syndrome)
  • Glucagonoma
  • Insulinoma
  • Somatostatinoma
  • VIPoma (Verner-Morrison syndrome)

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

What are the symptoms of Pancreatic Islet Cell Tumor?

Symptoms depend on which hormone is made by the tumor.

For example, insulinomas produce insulin, which lowers blood sugar level. Symptoms may include:

  • Feeling tired or weak
  • Shaking or sweating
  • Headache
  • Hunger
  • Nervousness, anxiety, or feeling irritable
  • Unclear thinking or feeling uneasy
  • Double or blurry vision
  • Fast or pounding heartbeat

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:

  • Abdominal pain
  • Diarrhea
  • Ulcers in the stomach and small bowel
  • Vomiting blood (occasionally)

Glucagonomas make the hormone glucagon, which helps the body raise blood sugar level. Symptoms can include:

  • Diabetes
  • Red, blistery rash in the groin or buttocks
  • Weight loss
  • Frequent urination and thirst

Somatostatinomas make the hormone somatostatin. Symptoms may include:

  • High blood sugar
  • Gallstones
  • Yellowish appearance to skin, and eyes
  • Weight loss
  • Diarrhea with foul smelling stools

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:

  • Severe diarrhea that may lead to dehydration
  • Low blood potassium levels, and high calcium levels
  • Abdominal cramps
  • Weight loss
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What are the current treatments for Pancreatic Islet Cell Tumor?

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.

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What are the support groups for Pancreatic Islet Cell Tumor?

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.

What is the outlook (prognosis) for Pancreatic Islet Cell Tumor?

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.

What are the possible complications of Pancreatic Islet Cell Tumor?

Complications of these tumors include:

  • Diabetes
  • Hormone crises (if the tumor releases certain types of hormones)
  • Severe low blood sugar (from insulinomas)
  • Severe ulcers in the stomach and small intestine (from gastrinomas)
  • Spread of the tumor to the liver
When should I contact a medical professional for Pancreatic Islet Cell Tumor?

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

How do I prevent Pancreatic Islet Cell Tumor?

There is no known prevention for these tumors.

Endocrine glands
Pancreas
What are the latest Pancreatic Islet Cell Tumor Clinical Trials?
Phase I Study of MEM-288 Oncolytic Virus in Solid Tumors Including Non-Small Cell Lung Cancer (NSCLC)

Summary: This phase I trial is designed as an open-label, dose escalation trial of MEM-288 monotherapy in which investigators aim to find the maximum tolerated dose (MTD) and recommended phase II dose (RP2D). Subjects with selected solid tumors including non-small cell lung cancer (NSCLC) who have a tumor lesion which is accessible for injection will undergo intratumoral injection of MEM-288. The study rat...

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Metabolomics and Genetic Diagnosing Pancreatic Neuroendocrine Tumors in Multiple Endocrine Neoplasia Type 1 Patients

Objectives: The aim of the present study is to assess the significance of metabolomics and genetics in diagnosing and survival evaluation for pNET in the periodic follow-up of MEN1 patients. Aim 1: To evaluate the relationship of serum global metabolic profiles with subsequent development of aggressive PNET and evaluate patients survival in a nested case-control study of MEN1 patients who have developed aggre...

What are the Latest Advances for Pancreatic Islet Cell Tumor?
A Case of Pancreatic Neuroendocrine Tumor G3 Which Responded Remarkably to Chemotherapy.
Primary neuroendocrine carcinoma of the breast with leptomeninges metastasis: A case report and literature review.
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Editorial.
Who are the sources who wrote this article ?

Published Date: January 25, 2022
Published 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.

What are the references for this article ?

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 June 21, 2022.

National Comprehensive Cancer Network website. NCCN clinical practice guidelines in oncology (NCCN guidelines). Neuroendocrine and adrenal tumors. Version 1.2022. www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf. UpdatedMay 23, 2022. Accessed June 24, 2022.

National Comprehensive Cancer Network website. NCCN guidelines for patients. Neuroendocrine tumors. 2022. www.nccn.org/patients/guidelines/content/PDF/neuroendocrine-patient.pdf. Accessed June 24, 2022.