This rare, often slow-growing tumor in the pancreas can produce hormones, causing various symptoms.
What is a pancreatic neuroendocrine tumor?
A pancreatic neuroendocrine tumor (PanNET) is a type of tumor that forms in the hormone-producing cells of the pancreas. The pancreas is an organ located behind the stomach that produces enzymes for digestion and hormones, such as insulin, that regulate blood sugar levels.
PanNETs are relatively rare compared to other types of pancreatic cancer. They develop from cells in the pancreas called islet cells, which are responsible for producing hormones. Some PanNETs produce excess hormones, leading to specific symptoms (functional tumors). Others don't produce hormones (non-functional tumors) and may not cause symptoms until they grow larger.
Because PanNETs are less common, data is challenging to collect and report. According to the American Cancer Society, pancreatic neuroendocrine tumors account for less than 2% of all cancers annually. Although they can occur at any age, peak diagnosis often occurs in a person’s 50s and 60s. Depending on the type of tumor, the stage, and treatment the survival rates for PanNETs are highly variable. AHN is here to provide individualized and comprehensive care for any person working through a PanNET diagnosis.
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Pancreatic neuroendocrine tumor symptoms and signs
Pancreatic neuroendocrine tumors (PanNETs) often present with hard-to-spot or nonspecific symptoms, making early detection challenging. Many symptoms can easily be mistaken for other, more common conditions, leading to delays in diagnosis. The specific symptoms a person experiences largely depend on whether the tumor is functional (producing excess hormones) or non-functional (not producing excess hormones). Talking with your doctor about any changes in your health, plus having your regular preventive care can be some of the best ways to stay on top of detecting PanNETs. Some symptoms to look for include:
Functional tumors produce excess hormones, causing distinct symptoms like:
- Insulinoma (excess insulin): Low blood sugar (shakiness, sweating, confusion, seizures), weight gain.
- Gastrinoma (excess gastrin): Peptic ulcers, heartburn, diarrhea, abdominal pain.
- Glucagonoma (excess glucagon): High blood sugar, skin rash, weight loss, blood clots.
- Somatostatinoma (excess somatostatin): Diabetes, gallstones, diarrhea, fatty stools.
- VIPoma (excess vasoactive intestinal peptide): Watery diarrhea, dehydration, muscle weakness.
Non-functional tumors don't overproduce hormones, so symptoms often appear later with tumor growth, including:
- Abdominal pain
- Jaundice (yellowing of skin and eyes)
- Unexplained weight loss
- Nausea and vomiting
- Loss of appetite
Causes and risk factors
The exact causes of pancreatic neuroendocrine tumors (PanNETs) are largely unknown. Unlike some cancers linked directly to lifestyle factors like smoking, the development of PanNETs appears to be more complex and likely involves a combination of genetic predisposition and possibly environmental factors, although definitive evidence for most environmental factors is lacking.
Genetic factors
There are some genetic factors that show a slight predisposition to PanNETs. Talk with your doctor about your health history to understand your risk.
- Family history: A family history of PanNETs or other endocrine tumors significantly increases the risk. Certain genetic syndromes, such as multiple endocrine neoplasia type 1 (MEN1) and von Hippel-Lindau (VHL) disease, are strongly associated with an increased risk of PanNETs and other endocrine tumors. These syndromes involve inherited genetic mutations that predispose individuals to the development of tumors in multiple endocrine glands.
- Specific gene mutations: Research is ongoing to identify specific gene mutations that may increase the risk of PanNETs, even outside of known genetic syndromes. Mutations in genes involved in cell growth and repair are likely implicated.
Risk factors
While the evidence is less conclusive than genetic factors, there is some indication that PanNETs can result from certain risk factors.
- Exposure to certain chemicals: While not definitively proven, some studies suggest a possible link between exposure to certain chemicals and an increased risk of PanNETs. More research is needed to confirm these associations.
- Certain medical conditions: Some chronic medical conditions have been suggested as potential risk factors, though evidence remains limited and often correlational, not necessarily causal.
- Age: The incidence of PanNETs increases with age, suggesting that the accumulation of genetic and/or environmental damage over time may play a role.
Factors NOT strongly linked
Despite these lifestyle choices not being strongly connected to PanNETs, it’s still important to quit smoking, have a healthy diet, and limit or abstain from alcohol.
- Smoking: Unlike many other cancers, there's no strong evidence linking smoking to an increased risk of PanNETs.
- Diet: While a healthy diet is always beneficial for overall health, there's no strong evidence to suggest a direct link between specific dietary factors and the risk of PanNETs.
- Alcohol consumption: The evidence for a link between alcohol consumption and PanNET risk is weak and inconclusive.
Pancreatic neuroendocrine tumor screening and diagnosis
Because Pancreatic NETs are often slow-growing and may not cause symptoms in early stages, they can be challenging to detect. There is no routine screening test for pancreatic NETs for the general population. However, screening might be considered for individuals with a high risk of developing these tumors, such as those with certain genetic syndromes. Diagnosis typically involves a combination of different screening tools.
Early diagnosis is crucial for improving the prognosis of pancreatic NETs. If you experience symptoms such as abdominal pain, jaundice, unexplained weight loss, or sudden-onset diabetes, it's important to consult a healthcare professional promptly.
Your doctor will inquire about your symptoms, medical history, and family history of endocrine tumors.
Blood and urine tests may reveal elevated levels of hormones secreted by the tumor, such as chromogranin A, gastrin, insulin, glucagon, or vasoactive intestinal peptide (VIP).
Imaging studies
Imaging studies play a crucial role in diagnosing and staging pancreatic neuroendocrine tumors (PanNETs). They help to locate the tumor, determine its size and extent, and assess whether it has spread to other parts of the body (metastasis). Different imaging techniques offer complementary information, and the choice of which tests to use depends on several factors, including the patient's symptoms and the suspected location of the tumor. AHN offers patients a full range of imaging study options paired with the expertise, compassionate care, and cutting-edge technology, found at AHN. This combination of technology and care creates a difference from clinics elsewhere. Options available at AHN include:
- Endoscopic Ultrasound (EUS): A thin, flexible tube with an ultrasound probe is inserted into your digestive tract to visualize the pancreas and nearby structures.
- CT scan: Provides detailed images of your internal organs to detect tumors and assess their size and location.
- MRI Scan: Uses a magnetic field and radio waves to create images of your organs and tissues, which can help distinguish between cancerous and noncancerous tumors.
- Gallium 68 Dotatate PET scan: This is a diagnostic imaging test that allows your doctor to identify PanNets.
- Octreoscan: A specialized scan that uses a radioactive tracer to identify NETs based on their tendency to overexpress somatostatin receptors.
Biopsy
While imaging studies are essential for detecting pancreatic masses, a biopsy is generally necessary for confirming a diagnosis of PanNET, determining its grade and type, and guiding treatment decisions. The choice of biopsy method will depend on several factors, including the tumor’s location, size, and the experience of the physician performing the procedure. A sample of tissue from the tumor is examined under a microscope to confirm the diagnosis and determine the tumor grade, which indicates its growth rate and likelihood of spreading.
Types and stages of pancreatic neuroendocrine tumor
Pancreatic NETs are categorized based on the hormones they produce, although some may not secrete hormones at all (non-functional tumors). It's important to discuss all treatment options and potential risks and benefits with your doctor. Regular follow-up appointments are crucial for monitoring tumor response, managing side effects, and addressing any new symptoms.
Common types include:
- Insulinomas: These tumors secrete insulin, leading to low blood sugar (hypoglycemia).
- Gastrinomas: Producing gastrin, a hormone that stimulates stomach acid production, this type of PanNET can potentially cause peptic ulcers and digestive issues (Zollinger-Ellison syndrome).
- Glucagonomas: These tumors secrete glucagon, which regulates blood sugar levels, potentially leading to high blood sugar (hyperglycemia) and skin rashes.
- Somatostatinomas: This type of tumor releases somatostatin, which inhibits the release of other hormones, leading to a variety of symptoms depending on the affected hormones.
- VIPomas: These tumors produce vasoactive intestinal peptide (VIP), leading to severe watery diarrhea, dehydration, and electrolyte imbalances.
- Non-functional tumors: While these tumors do not secrete hormones, they are typically diagnosed when they have grown large enough to cause symptoms due to their size or pressure on nearby organs.
The staging of pancreatic NETs describes the extent of tumor growth and spread. It helps determine prognosis and treatment options.
The most common staging system is the TNM system:
- T (Tumor) describes the size and extent of the primary tumor within the pancreas:
- T1: Tumor confined to the pancreas, less than 2 cm
- T2: Tumor confined to the pancreas, more than 2 cm
- T3: Tumor extends beyond the pancreas but not into major blood vessels
- T4: Tumor invades major blood vessels or other nearby organs
- N (Nodes): Indicates whether the cancer has spread to nearby lymph nodes.
- N0: No lymph node involvement
- N1: Spread to regional lymph nodes
- M (Metastasis): Describes whether the cancer has spread to distant organs (metastasized).
- M0: No distant metastasis
- M1: Distant metastasis present
Based on the TNM system, pancreatic NETs are assigned a stage, ranging from I (early stage) to IV (advanced stage). The stage reflects the tumor's aggressiveness and guides treatment decisions.
Pancreatic neuroendocrine tumor treatment
Treatment options at AHN include some of the most comprehensive and cutting-edge available. Patients will experience a multidisciplinary team and personalized plan that is specific to their diagnosis. Options may include a combination of different techniques and treatment.
Surgery
Often the first-line approach for localized tumors that haven't spread. Potential for cure if the tumor is completely removed.
- Enucleation: Removal of the tumor only.
- Partial pancreatectomy: Removal of the part of the pancreas containing the tumor.
- Total pancreatectomy: Removal of the entire pancreas (usually requires lifelong insulin therapy).
Radiation therapy
At AHN, radiation therapy, including external beam radiation and, in some cases, brachytherapy , can help shrink pancreatic neuroendocrine tumors (PanNETs), relieving symptoms caused by tumor pressure. It may be used alone or in combination with other treatments like surgery or systemic therapy, especially for locally advanced or metastatic disease. Radiation therapy's effectiveness in PanNETs is often less pronounced than in other cancer types.
Medications
Depending on the type of tumor and its staging, medications may be a useful tool in treating pancreatic neuroendocrine tumors. Your doctor and care team will evaluate the most effective combination to help treat the tumor. Some options include:
- Somatostatin analogs (SSAs): Block hormone production and slow tumor growth such as octreotide and lanreotide.
- Peptide Receptor Radionuclide Therapy (PRRT): PRRT offers a targeted approach to treating certain neuroendocrine tumors. By binding to specific receptors on tumor cells, PRRT delivers a precise, low-dose radiation treatment that effectively slows or stops tumor growth. While not a cure, PRRT significantly improves both the length and quality of life for many patients.
- Targeted therapies: Interfere with specific pathways involved in tumor growth including everolimus, sunitinib, and cabozantionib.
- Chemotherapy: Kills rapidly dividing cells, including cancer cells. May be used for tumors that are aggressive or cannot be removed surgically.
Symptom management
Using the multi-disciplinary and compassionate care at AHN, we will help patients manage any symptoms so they can get back to the lives they enjoy with support and resources.
- Medications: To control symptoms caused by hormone overproduction, such as diarrhea, low blood sugar, or stomach acid.
- Dietary changes: To manage symptoms and support overall health.
Learn more about AHN Cancer Support Services and how we can help you manage your care before, during and after treatment.
Pancreatic neuroendocrine tumor FAQs
There’s a lot to understand about pancreatic neuroendocrine tumors. We’re here to help, if you don’t see your question answered below. Reach out and we will talk with you to help you feel better informed.
What is neuroendocrine cancer?
Neuroendocrine cancers are rare tumors that originate in neuroendocrine cells, which are specialized cells found throughout the body that act as messengers between the nervous and endocrine systems. These cells produce hormones, and the tumors can therefore release excess hormones, causing a variety of symptoms depending on their location. Treatment varies depending on the cancer's location, stage, and whether it's functioning (hormone-producing) or non-functioning.
What is a neuroendocrine tumor?
Neuroendocrine tumors are slow-growing tumors that arise from neuroendocrine cells, which are specialized cells found throughout the body that produce hormones and other signaling molecules. These tumors can occur in various organs, including the lungs, pancreas, gastrointestinal tract, and others. Depending on their location and whether they produce excess hormones, NETs can present with a wide range of symptoms or may be asymptomatic.
Contact us
Call the AHN Cancer Help Line anytime at (412) NURSE-4-U (412) 687-7348 to schedule a cancer-related appointment or to just talk with our nurses about diagnoses, treatments, and side effects.