Pancreatic neuroendocrine tumors
Make an Appointment
Talk to DoctorOverview
Pancreatic neuroendocrine tumor (pNET) (also known as islet cell tumor) is a rare type of hormone-producing tumors that form in tissue of pancreas. The pancreas is a gland that is located in front of the spine and behind the stomach. It is a gland which plays a role in the digestive system by producing enzymes, which help to break down food as well as regulate blood sugar levels.
Pancreatic neuroendocrine tumor can be noncancerous (benign) or cancerous (malignant). Both types can result in major health issues. When pancreatic neuroendocrine tumor is cancerous, they are called pancreatic endocrine cancer (islet cell carcinoma).
Treatment option of pancreatic neuroendocrine tumors are surgery, peptide receptor radionuclide therapy, targeted therapy, radiofrequency ablation, and chemotherapy.
Classification of pancreatic neuroendocrine tumors
- Non-functional pancreatic NET: is a tumor that does not produce extra hormone and usually do not cause symptoms until they increase in size and out pressure on nearby tissue.
- Functional pancreatic NET: is a tumor that produce excessive amounts of hormones. The clinical manifestations depend on the hormone that it produces. Functional pancreatic NET includes the following:
- Gastrinoma: is a tumor that develops in cells that produce gastrin. Gastrin is a hormone which is used in food digestion.
- Insulinoma: is a tumor that produces an excessive amount of insulin, which cause hypoglycemia (low blood sugar).
- Glucagonoma: is a tumor that produces an excessive amount of glucagon, which causes hyperglycemia (high blood sugar). The glucagon is a hormone that work with insulin to regulate the blood sugar level.
Symptoms
Non-functional pancreatic NET
The symptoms associated with this type depend solely on their size. The following are the signs and symptoms of this condition include:
- Jaundice (yellowish discoloration of the eyes and skin)
- Abdominal pain
- Diarrhea
- Nausea
- Loss of appetite
- Unexplained weight loss
Functional pancreatic NET
The symptoms depend on the hormone they produce. The following are signs and symptoms of this condition includes:
- Gastrinoma:
- Abdominal pain
- Heartburn
- Diarrhea
- Insulinoma:
- Hypoglycemia (low blood sugar)
- Weakness and tiredness
- An increase heartbeat
- Confusion
- Loss of consciousness
- Blurred vision
- Glucagonoma:
- Skin rash
- Unexplained weight loss
- Sore around the mouth
- Passing a lot of urine
- Diarrhea
Causes
Although most pancreatic neuroendocrine tumors (NETs) have an unknown specific cause, however researchers have identified many risk factors that can increase a person’s chance of developing this condition.
When hormone-producing islet cells in the pancreas undergo changes in their DNA, pancreatic neuroendocrine tumors develop. These instructions vary resulting in genetic mutations. The cells may start to multiply out of control and subsequently form a tumor, which is a collection of malignant cells.
Risk factors
The following factors have been linked to an increased risk of pancreatic neuroendocrine tumors:
- Gender - These tumors are significantly more common in male than in female.
- Family history – The risk is high if a family member has been diagnosed with a pancreatic neuroendocrine tumor.
- Inherited genetic syndromes – Some pancreatic NET may be linked to an inherited genetic syndrome such as:
- Multiple endocrine neoplasia, type 1 (MEN 1) syndrome: MEN1 is a rare genetic disorder most often caused by a mutation in the MEN1 gene. This syndrome can affect parathyroid, pituitary gland, and the islet cells of the pancreas. These tumors typically develop early in life and are typically non-functioning pancreatic NET.
- Von Hippel-Lindau disease: A small number of pancreatic NETs, which typically appear at younger ages (as low as 20 years old), are brought on by variations in the VHL gene. These tumors often slowly growing and are not functional.
- Neurofibromatosis type1: also called Von Recklinghausen’s disease, is a genetic disorder caused by mutation in NF1 gene. People with this syndrome increase risk of pancreatic NET.
Diagnosis
Tests and procedures used to diagnose pancreatic neuroendocrine tumors include:
- Blood tests: Pancreatic NETs can frequently be diagnosed using blood tests that measure the levels of specific pancreatic hormones.
- Urine test: This method is used to identify breakdown products of hormones processing in your body.
Imaging tests: - Computer tomography (CT) scans: create detailed images of the organs. The pancreas may be plainly seen during this examination, which also determines whether the pancreatic NET has progressed to a nearby lymph nodes or other organs including the liver. Additionally, it can be utilized as a guide to needle biopsy when the location is difficult.
- Magnetic resonance imaging (MRI): The body’s soft tissues can be seen in great detail on an MRI scan. However, MRI scans substitute radio waves and powerful magnets for X-rays. In order to observe details more clearly during the scan, dyes may be injected into a vein beforehand. Sometimes, an MRI scan can detect cancer metastasis to the liver more accurately than a CT scan.
- Endoscopic Ultrasound: An endoscope with a tiny ultrasonic probe on the end is used for this test. Your pancreas is inspected using a specialized ultrasound. In order to get biopsy samples of a tumor, a tiny, hollow needle can be inserted through the endoscope.
- Surgical biopsy: Specialists may recommend minimally invasive surgery in some circumstances in order to collect a tissue sample for testing (biopsy). A small camera and specialized tools are inserted during a laparoscopy by the surgeon through a series of tiny incisions in your abdomen.
Treatment
The treatment for pancreatic neuroendocrine tumor depends on the cell types involved in the tumor, the size and other characteristics.
Surgery
Surgery is usually the primary treatment for a pancreas NET that is localized.
- Distal pancreatectomy: is surgery procedure to remove the body and tail of the pancreas. This procedure is usually performed in cases where the pancreas’s tail is affected by cancer.
- Whipple procedure (pancreaticoduodenectomy) – The Whipple procedure is an operation procedure which will remove the head of the pancreas, the duodenum (the first part of small intestine), the gallbladder and portion of the bile duct. The remaining parts will be reconnected to remain food digestion track. This procedure commonly used for tumor in the head of the pancreas.
Peptide receptor radionuclide therapy (PRRT)
This is the radioactive substance that is injected into a vein together with a medication that kills cancerous cells. The drug binds to the body’s pancreatic neuroendocrine tumor cells. The drug directly delivers radiation to the cancer cells over the course of days to weeks, resulting in death of the tumor.
Targeted therapy
This therapy uses drugs that specifically targets the cancer cells. Certain advanced or recurrent pancreatic neuroendocrine tumors are treated with targeted therapy
Radiofrequency ablation
This method utilizes energy waves to heat up and kill cancer cells.
Treatment for cancer that spreads to the liver
There are numerous treatments available for pancreatic neuroendocrine tumors, which typically metastasize to the liver. Hepatic artery occlusion, chemotherapeutic embolization, radioembolization, and liver transplantation are among the available treatments for liver tumors. Other options include treatment to stop blood flow to the tumors (hepatocellular occlusion).
Chemotherapy
This method uses chemicals combined in the medication to attack cancer cells and to destroy them. It can be used together along with surgery.
