Pancreatic neuroendocrine tumors
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 tumorPancreatic neuroendocrine tumors (NET) are rare tumors of the pancreas that start from a type of cell called the islet cell.
Tumors
A tumor is an abnormal growth of body tissue. Tumors can be cancerous (malignant) or noncancerous (benign).
Read Article Now Book Mark ArticleCauses
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 NETs can be noncancerous (benign) or cancerous (malignant).
Benign
Benign refers to a condition, tumor, or growth that is not cancerous. This means that it does not spread to other parts of the body. It does not in...
Read Article Now Book Mark ArticleMalignant
The term "malignancy" refers to the presence of cancerous cells that have the ability to spread to other sites in the body (metastasize) or to invade...
Read Article Now Book Mark ArticleNETs include:
- Gastrinoma (Zollinger-Ellison syndrome)
Zollinger-Ellison syndrome
Zollinger-Ellison syndrome is a condition in which the body produces too much of the hormone gastrin. Most of the time, a small tumor (gastrinoma) i...
Read Article Now Book Mark Article - Glucagonoma
Glucagonoma
Glucagonoma is a very rare tumor of the islet cells of the pancreas, which leads to an excess of the hormone glucagon in the blood.
Read Article Now Book Mark Article - Insulinoma
Insulinoma
An insulinoma is a tumor in the pancreas that produces too much insulin.
Read Article Now Book Mark Article - Somatostatinoma
- VIPoma (Verner-Morrison syndrome)
Verner-Morrison syndrome
VIPoma is a very rare cancer that usually grows from cells in the pancreas called islet cells.
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A family history of multiple endocrine neoplasia, type I (MEN I) is a risk factor for the development of NETs.
Endocrine
Endocrine glands release (secrete) hormones into the bloodstream. The endocrine glands include:AdrenalHypothalamusIslets of Langerhans in the pancrea...
Read Article Now Book Mark ArticleMEN I
Multiple endocrine neoplasia (MEN) type I is a disease in which one or more of the endocrine glands are overactive or forms a tumor. It is passed do...
Read Article Now Book Mark ArticleSymptoms
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 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
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:
- Fasting glucose level
Fasting glucose
A blood sugar test measures the amount of a sugar called glucose in a sample of your blood. Glucose is a major source of energy for most cells of the...
Read Article Now Book Mark Article - Gastrin level
Gastrin level
The gastrin blood test measures the amount of the hormone gastrin in blood.
Read Article Now Book Mark Article - Glucose tolerance test
Glucose tolerance test
The glucose tolerance test is a lab test to check how your body moves sugar from the blood into tissues like muscle and fat. The test is often used ...
Read Article Now Book Mark Article - Secretin stimulation test for pancreas
Secretin stimulation test
The secretin stimulation test measures the ability of the pancreas to respond to a hormone called secretin. The small intestine produces secretin wh...
Read Article Now Book Mark Article - Blood glucagon level
Blood glucagon
A glucagon blood test measures the amount of a hormone called glucagon in your blood. Glucagon is produced by specific cells in the pancreas. It he...
Read Article Now Book Mark Article - Blood insulin C-peptide level
Blood insulin C-peptide
C-peptide is a substance that is created when the hormone insulin is produced and released into the body. The insulin C-peptide test measures the am...
Read Article Now Book Mark Article - Blood insulin level
- Fasting serum somatostatin level
- Serum vasoactive intestinal peptide (VIP) level
Imaging tests may be done:
- Abdominal CT scan
Abdominal CT scan
An abdominal CT scan is an imaging method. This test uses x-rays to create cross-sectional pictures of the belly area. CT stands for computed tomog...
Read Article Now Book Mark Article - Abdominal ultrasound
Abdominal ultrasound
Abdominal ultrasound is a type of imaging test. It is used to look at organs in the abdomen, including the liver, gallbladder, spleen, pancreas, and...
Read Article Now Book Mark Article - Endoscopic ultrasound
Ultrasound
Ultrasound uses high-frequency sound waves to make images of organs and structures inside the body.
Read Article Now Book Mark Article - MRI of abdomen
MRI of abdomen
An abdominal magnetic resonance imaging scan is an imaging test that uses powerful magnets and radio waves. The waves create pictures of the inside ...
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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 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 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.
Cancer support group
The following organizations are good resources for information on cancer:American Cancer Society. Support and online communities. www. cancer. org/...
Read Article Now Book Mark ArticleOutlook (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:
- 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 to Contact a Medical Professional
Contact 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.
References
Khreiss 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.
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Pancreas - illustration
The pancreas is an elongated, tapered gland that is located behind the stomach and secretes digestive enzymes and the hormones insulin and glucagon.
Pancreas
illustration
Endocrine glands - illustration
Endocrine glands release hormones (chemical messengers) into the bloodstream to be transported to various organs and tissues throughout the body. For instance, the pancreas secretes insulin, which allows the body to regulate levels of sugar in the blood. The thyroid gets instructions from the pituitary to secrete hormones which determine the rate of metabolism in the body (the more hormone in the bloodstream, the faster the chemical activity; the less hormone, the slower the activity).
Endocrine glands
illustration
Pancreas - illustration
The pancreas is an elongated, tapered gland that is located behind the stomach and secretes digestive enzymes and the hormones insulin and glucagon.
Pancreas
illustration
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.