Overview
Pancreatic neuroendocrine tumors (NETs) are a rare type of cancer that originates from the hormone-producing cells in the pancreas called islet cells. They can be functional, producing excess hormones, or nonfunctional, not producing extra hormones. Symptoms can include dizziness, blurred vision, headaches, increased thirst and hunger, heartburn, weakness, fatigue, muscle cramps, indigestion, diarrhea, weight loss, skin rash, constipation, pain in the abdomen or back, yellowing of the skin and eyes, and more. The exact cause of pancreatic neuroendocrine tumors is not clear, but DNA changes in islet cells are known to play a role. Risk factors include a family history of pancreatic neuroendocrine tumors, inherited syndromes like MEN1, VHL, NF1, and tuberous sclerosis, which increase the risk due to changes in DNA. If you have any symptoms that worry you, it is important to consult with a healthcare professional.
Understanding the disease
Symptoms
The symptoms of pancreatic neuroendocrine tumors can vary depending on the specific type of tumor. Some common symptoms include heartburn, weakness, fatigue, muscle cramps, indigestion, diarrhea, weight loss, skin rash, constipation, pain in the abdomen or back, yellowing of the skin and the whites of the eyes, dizziness, blurred vision, headaches, increased thirst and hunger. If you experience any of these symptoms and they worry you, it's important to make an appointment with a healthcare professional.
Risk Factors
Risk factors for developing pancreatic neuroendocrine tumors include a family history of pancreatic neuroendocrine tumors, as well as certain inherited syndromes that increase the risk of tumors, such as multiple endocrine neoplasia, type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1), and tuberous sclerosis. These inherited syndromes are caused by changes in the DNA that allow cells to grow and divide more than needed.
Development
Pancreatic neuroendocrine tumors develop when cells in the pancreas, specifically the hormone-producing islet cells, undergo changes in their DNA. These changes, or mutations, cause the cells to multiply quickly and continue living beyond their normal life cycle. This results in the formation of extra cells that may form a mass or tumor. Some pancreatic neuroendocrine tumors produce excess amounts of specific hormones, while others do not. Examples of functional tumors include insulinoma, gastrinoma, and glucagonoma, which produce too much insulin, gastrin, or glucagon, respectively. Nonfunctional tumors, on the other hand, do not produce extra hormones. It is not clear what causes the DNA changes that lead to pancreatic neuroendocrine tumor development. However, some factors may increase the risk, such as a family history of pancreatic neuroendocrine tumors, inherited syndromes like multiple endocrine neoplasia, type 1 (MEN 1), von Hippel-Lindau (VHL) disease, neurofibromatosis 1 (NF1), and tuberous sclerosis, and certain genetic conditions.
Assessment and Diagnosis
Triage
If you have symptoms such as heartburn, weakness, fatigue, muscle cramps, indigestion, diarrhea, weight loss, skin rash, constipation, pain in the abdomen or back, yellowing of the skin and the whites of the eyes, dizziness, blurred vision, headaches, or increased thirst and hunger, and they worry you, you should make an appointment with a health care professional.
Diagnosis
To diagnose pancreatic neuroendocrine tumors, the following tests and procedures may be used:
- Blood tests: These tests may show excess hormones or other signs of a pancreatic neuroendocrine tumor. Blood samples can also be used to look for DNA changes that signal an increased risk of these tumors.
- Urine tests: A test of your urine may show breakdown products resulting from the body's processing of hormones.
- Imaging tests: Imaging tests, such as X-ray, MRI, CT, and positron emission tomography (PET) scan, can show the location and size of a pancreatic neuroendocrine tumor. Remember that the specific diagnostic tests may vary depending on the individual case and the location of the tumor. It's essential to consult with a healthcare professional for a proper diagnosis and treatment plan.
Management and Treatment
Complications
Potential complications of pancreatic neuroendocrine tumors may include:
- Hormonal imbalances: The tumor may overproduce certain hormones, leading to imbalances in the body. This can cause a variety of symptoms, depending on the specific hormone involved.
- Nausea and vomiting: Some pancreatic neuroendocrine tumors can cause hormones that irritate the stomach, leading to nausea and vomiting.
- Jaundice: Tumors that obstruct the bile ducts can cause jaundice, leading to yellowing of the skin and eyes.
- Pancreatitis: Tumors can cause inflammation of the pancreas, leading to pancreatitis. Symptoms include severe abdominal pain, nausea, and fever.
- Bleeding: Tumors can cause internal bleeding, potentially leading to anemia and other complications.
- Spread (metastasis) to other organs: Pancreatic neuroendocrine tumors can spread to other organs, such as the liver, lungs, and bones. This can lead to complications specific to the affected organ, as well as overall decreased health and well-being.
- Malnutrition: Pancreatic neuroendocrine tumors can interfere with the proper absorption of nutrients, leading to malnutrition and weight loss. These complications can vary depending on the specific type, location, and stage of the tumor. It is important to discuss any concerns with a healthcare professional.
Treatment
Treatment options for pancreatic neuroendocrine tumors may include surgery, chemotherapy, targeted drug therapy, and peptide receptor radionuclide therapy (PRRT). Surgery is typically used to remove the tumor, and may involve distal pancreatectomy or the Whipple procedure. Chemotherapy and targeted drug therapy may be recommended after surgery to reduce the risk of recurrence or for advanced tumors that cannot be removed with surgery. PRRT is a form of therapy that combines a medicine targeting cancer cells with a radioactive substance to deliver radiation directly to the tumor cells. The choice of treatment depends on the type, stage, and location of the tumor.
Preparing for medical consultation
To prepare for an appointment for pancreatic neuroendocrine tumors, you should:
- Make an appointment with your primary care provider or a specialist, such as a gastroenterologist or an oncologist.
- Gather your medical records related to your condition and bring them to your appointment.
- Create a list of your symptoms, including when they started and how often they occur.
- Prepare a list of key personal information, such as major stresses, recent life changes, and family medical history.
- Bring a list of all medicines, vitamins, or other supplements you take, including the doses.
- Compile a list of questions to ask your healthcare team, such as the causes of your symptoms, recommended tests, best course of action, alternative treatments, potential consequences of not having medical treatment, and how to manage other health conditions. Consider bringing a family member or friend to your appointment for support and to help remember the information provided. Remember to ask for any brochures or printed materials and inquire about recommended websites for further information.