Overview
Neuroendocrine neoplasms of the pancreas (also called pancreatic NETs, pNETs, or islet cell tumors) are a group of rare tumors that grow from the hormone-producing cells of the pancreas. Unlike the more common type of pancreatic cancer (pancreatic ductal adenocarcinoma), pNETs tend to grow more slowly and often have a better outlook. The pancreas normally makes hormones like insulin and glucagon that control blood sugar, as well as digestive enzymes. When a neuroendocrine tumor develops, it can either silently grow without making extra hormones (called non-functional) or it can overproduce hormones and cause specific symptoms (called functional). Symptoms depend on whether the tumor is functional or not. Non-functional tumors may cause no symptoms until they grow large enough to press on nearby organs, leading to belly pain, weight loss, or jaundice (yellowing of the skin). Functional tumors cause symptoms related to the hormone they produce — for example, insulinomas cause dangerously low blood sugar, while gastrinomas cause severe stomach ulcers. Other symptoms can include diarrhea, skin flushing, and fatigue. Treatment depends on the tumor's size, grade, and whether it has spread. Options include surgery (which can sometimes cure the disease), targeted drug therapies like everolimus (Afinitor) and sunitinib (Sutent), somatostatin analogs like octreotide (Sandostatin) and lanreotide (Somatuline), and a radioactive treatment called PRRT (peptide receptor radionuclide therapy, brand name Lutathera). Many patients live for years or even decades with this disease, especially when it is caught early.
Also known as:
Key symptoms:
Belly pain or discomfortUnexplained weight lossYellowing of the skin or eyes (jaundice)Episodes of very low blood sugar (shakiness, sweating, confusion) — in insulinomaSevere or recurring stomach ulcers — in gastrinomaWatery diarrheaSkin flushing (redness of the face and neck)Fatigue and weaknessNausea or vomitingNew-onset diabetes or worsening blood sugar controlA lump or mass felt in the abdomenLoss of appetite
Variable
Can be inherited in different ways depending on the underlying gene
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
6 eventsGCS Ramsay Santé pour l'Enseignement et la Recherche — NA
Qilu Hospital of Shandong University
Australasian Gastro-Intestinal Trials Group — PHASE2
Ruijin Hospital — NA
Peking Union Medical College Hospital
Oslo University Hospital — NA
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Neuroendocrine neoplasm of pancreas.
2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Neuroendocrine neoplasm of pancreas.
Community
No community posts yet. Be the first to share your experience with Neuroendocrine neoplasm of pancreas.
Start the conversation →Latest news about Neuroendocrine neoplasm of pancreas
Disease timeline:
New recruiting trial: Endoscopic Ultrasound Guided Radio Frequency Ablation of Pancreatic Neuroendocrine Neoplasms
A new clinical trial is recruiting patients for Neuroendocrine neoplasm of pancreas
New recruiting trial: Cessation of Somatostatin Analogues After PRRT in Mid, Hind-Gut and Pancreatic Neuroendocrine Tumours
A new clinical trial is recruiting patients for Neuroendocrine neoplasm of pancreas
New recruiting trial: In Vitro Organoid Drug Sensitivity-Guided Treatment for Advanced Pancreatic Neuroendocrine Tumor
A new clinical trial is recruiting patients for Neuroendocrine neoplasm of pancreas
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What is the grade and stage of my tumor, and what does that mean for my outlook?,Is surgery an option for me, and could it cure my disease?,Should I be tested for a hereditary syndrome like MEN1, and should my family members be tested too?,What treatment do you recommend first, and what are the main side effects I should expect?,Am I a candidate for PRRT (Lutathera), and how do I access it?,How often will I need scans and blood tests to monitor my disease?,Are there clinical trials I should consider?
Common questions about Neuroendocrine neoplasm of pancreas
What is Neuroendocrine neoplasm of pancreas?
Neuroendocrine neoplasms of the pancreas (also called pancreatic NETs, pNETs, or islet cell tumors) are a group of rare tumors that grow from the hormone-producing cells of the pancreas. Unlike the more common type of pancreatic cancer (pancreatic ductal adenocarcinoma), pNETs tend to grow more slowly and often have a better outlook. The pancreas normally makes hormones like insulin and glucagon that control blood sugar, as well as digestive enzymes. When a neuroendocrine tumor develops, it can either silently grow without making extra hormones (called non-functional) or it can overproduce hor
At what age does Neuroendocrine neoplasm of pancreas typically begin?
Typical onset of Neuroendocrine neoplasm of pancreas is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Neuroendocrine neoplasm of pancreas?
Yes — 2 recruiting clinical trials are currently listed for Neuroendocrine neoplasm of pancreas on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Neuroendocrine neoplasm of pancreas?
25 specialists and care centers treating Neuroendocrine neoplasm of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.