About Pancreatic Neuroendocrine Tumor
Pancreatic neuroendocrine tumors (PNETs) are rare tumors that arise from the neuroendocrine cells in the pancreas. The exact causes of PNETs are not fully understood, but certain genetic mutations and alterations have been associated with their development. These include mutations in genes such as MEN1, DAXX/ATRX, and TSC2.
Non-modifiable risk factors (factors a person cannot change or control) for PNET include:
- Family history: People with a family member diagnosed with PNET have an increased risk for developing PNET.
- Inherited syndromes: Certain syndromes that are passed from parent to child (inherited) increase the risk of PNET. These include multiple endocrine neoplasia type 1 (MEN1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1), and tuberous sclerosis.
Modifiable risk factors (factors a person potentially may be able to change or control) for PNET include:
- Smoking: Heavy smoking increases the risk of developing PNET.
- Alcohol: Heavy alcohol consumption increases the risk of PNET. Heavy alcohol use increases the risk of chronic pancreatitis, which is also a risk factor for PNET.
- Diabetes: PNET is more common in people with Type 2 diabetes compared to people who do not have diabetes.
- Chronic pancreatitis: Chronic pancreatitis increases the risk of PNET.
While these risk factors have been identified, they do not guarantee the development of PNETs. Conversely, the absence of these risk factors does not guarantee protection against PNETs. If you have concerns about your risk for PNETs, it is recommended to consult with a healthcare professional who can provide personalized advice based on your individual situation.
Symptoms depend on the on the type of pancreatic neuroendocrine tumors (PNETs) but commonly include:
- Flushing of in the face and neck
- Wheezing and shortness of breath
- Abdominal cramping with diarrea
- Nausea and vomiting
- Loss of appetite
- Unexplained weight loss
- Fatigue
- Indigestion
- Yellowing of the skin and eyes (jaundice)
Additional symptoms may occur based upon the type of hormone released by the PNET.
- Gastrinomas: These PNETs form in cells that make gastrin, a hormone needed for digestion of food. Symptoms of too much gastrin include:
- Stomach ulcers that keep recurring (coming back)
- Abdominal pain that spreads to the back
- Gastroesophageal reflux (flow of stomach contents back into the esophagus)
- Diarrhea
- Insulinomas: These PNETs form in cells that make insulin, a hormone that controls sugar (glucose) in the blood. Symptoms of too much insulin include:
- Low blood sugar: Symptoms include blurry vision, sweating, confusion, and feeling lightheaded, shaky or weak
- Fast heart rate
- Glucagonomas: These PNETs form in cells that make glucagon, a hormone that increases the amount of glucose in the blood to keep blood sugar from getting too low. Symptoms of too much glucagon include:
- Skin rash on face, stomach or legs
- High blood sugar: Symptoms include frequent urination, dry mouth, heaches, thirst
- Blood clots, including in the arms, legs, or lungs
- Sores at the corners of the mouth or sore tongue
- Muscle weakness, muscle cramps, tingling and numbness due to low potassium
- VIPomas: These PNETs release vasoactive intestinal peptide (VIP), a hormone that relaxes stomach and intestinal muscles and controls the sugar, salt, and water in the digestive tract. Too much VIP may cause:
- Watery diarrhea
- Dehydration
- Abdominal cramps or pain
- Somatostatinomas: These PNETs release somatostatin, a hormone that inhibits (blocks) the release of other hormones. Too much somatostatin causes:
- High blood sugar
- Diarrhea
- Fatty stool (steatorrhea)
- Gallstones
It's important to note that these symptoms are not exclusive to PNET and can be caused by other conditions as well. If you experience any of these symptoms, it's best to consult with a healthcare professional for a proper diagnosis and appropriate treatment.
To diagnose a pancreatic neuroendocrine tumors (PNETs), healthcare providers may perform the following examinations, tests, and procedures:
- Physical exam: The healthcare provider will look for physical signs of a medical problem, such as pain, swelling, or the presence of masses in your body.
- Lab tests: Blood tests may be ordered to check for increased levels of hormones and glucose in your bloodstream, which can indicate a PNET.
- Imaging studies: Your healthcare provider may recommend imaging tests to visualize your pancreas and look for the presence of a tumor. These tests may include ultrasound, magnetic resonance imaging (MRI), or computed tomography (CT) scan.
- Biopsy: In some cases, your doctor may perform a minor exploratory surgery to obtain a small sample of tissue from your pancreas for a biopsy. This helps confirm the diagnosis.
To determine the stage or severity of the PNET, additional examinations, tests, and procedures may include:
- Additional imaging studies: Further imaging studies such as PET scans or somatostatin receptor scintigraphy (SRS) may be performed to assess the extent of tumor spread and identify metastasis.
- Genetic testing: Some PNETs are associated with inherited genetic syndromes. Genetic testing can help identify these syndromes and guide treatment decisions.
- Endoscopic ultrasound (EUS): This procedure combines endoscopy with ultrasound to obtain detailed images of the pancreas and surrounding structures. It can help determine the size and location of the tumor.
- Angiography: In certain cases, angiography may be used to evaluate blood flow to the tumor and help plan treatment options.
It's important to discuss these examinations, tests, and procedures with your healthcare provider to determine which ones are appropriate for your individual situation.
The goals of treatment for pancreatic neuroendocrine tumors (PNETs) depend on various factors such as tumor type, size, and stage. The main treatment goals for PNET are to remove the tumor completely or decreasing tumor size, and control symptoms.
Treatment options for PNETs include:
- Surgery: Surgery is the primary treatment option for localized tumors. It involves removing the tumor and surrounding tissue to prevent further spread. In some cases, endoscopic surgery may be suitable depending on the tumor's location, size, and malignancy degree.
- Chemotherapy: Chemotherapy uses drugs to kill tumor cells. It may be used for advanced cases where the tumor cannot be removed by surgery.
- Targeted therapy: Peptide receptor radionuclide therapy (PRRT) is a targeted therapy that can be used for neuroendocrine tumors. It specifically targets neuroendocrine tumor cells by attaching radioactive particles to them, which can kill the cancer cells.
- Locoregional therapy: This includes procedures like hepatic arterial occlusion or chemoembolization, which target liver metastases specifically.
- Hormone therapy: Medications may be given to remove hormones or blocks their action to stop cancer cells from growing. These medications are typically administered as injections every 28 days.
It's important to note that specific treatment recommendations depend on individual factors and should be discussed with a healthcare professional. Medication dosing may be affected by many factors, so it's crucial to consult with your healthcare professional about dosing for your individual situation. Other side effects can occur, so it's important to check with your healthcare professional or read the information provided with your medication for additional side effect information.