Overview
A functioning neuroendocrine tumor of the pancreas (also called a functional pancreatic NET, or F-pNET) is a rare type of tumor that grows in the pancreas — a gland in your belly that helps with digestion and controls blood sugar. What makes these tumors special is that they are 'functioning,' meaning they actively release hormones into your bloodstream. Depending on which hormone is released, the tumor has a specific name — for example, insulinoma (releases insulin), gastrinoma (releases gastrin), glucagonoma, VIPoma, or somatostatinoma. These extra hormones cause a wide range of symptoms that can affect many parts of the body. Because these tumors produce hormones, symptoms often appear before the tumor itself is found. For example, an insulinoma causes dangerously low blood sugar, while a gastrinoma causes severe stomach ulcers. This can make diagnosis tricky, and many patients see multiple doctors before getting the right answer. Treatment depends on the type and stage of the tumor. Surgery to remove the tumor is the main goal when possible and can be curative. When surgery is not an option, doctors use medications to control hormone levels and slow tumor growth. FDA-approved treatments include somatostatin analogs like octreotide (Sandostatin) and lanreotide (Somatuline), as well as everolimus (Afinitor) and sunitinib (Sutent) for advanced cases. Peptide receptor radionuclide therapy (PRRT) with lutetium-177 dotatate (Lutathera) is also approved for certain patients. With proper treatment, many people live for years with this condition.
Also known as:
Key symptoms:
Low blood sugar episodes (shakiness, sweating, confusion) — mainly with insulinomaSevere or recurring stomach ulcers — mainly with gastrinomaPersistent diarrheaSkin rash, especially a red, blistering rash on the legs and face — mainly with glucagonomaUnexplained weight lossAbdominal pain or discomfortNausea and vomitingWatery diarrhea and low potassium levels — mainly with VIPomaHigh blood sugar (diabetes-like symptoms) — mainly with glucagonoma or somatostatinomaFlushing or redness of the faceFatigue and weaknessGallstones — mainly with somatostatinomaHeartburn or acid reflux that does not respond to usual treatments
Variable
Can be inherited in different ways depending on the underlying gene
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
4 eventsITM Solucin GmbH — PHASE3
National Cancer Institute (NCI) — PHASE3
ITM Solucin GmbH — PHASE3
Mayo Clinic
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Functioning neuroendocrine tumor of pancreas.
3 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →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 Functioning neuroendocrine tumor of pancreas.
Community
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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 type of functioning pancreatic NET do I have, and which hormone is it producing?,Has the tumor spread, and am I a candidate for surgery?,Should I be tested for a hereditary condition like MEN1, and should my family members be tested?,What medications will I need, and what are the side effects?,Am I eligible for PRRT (Lutathera) or any clinical trials?,How often will I need imaging and blood tests to monitor the tumor?,What dietary changes should I make to manage my symptoms?
Common questions about Functioning neuroendocrine tumor of pancreas
What is Functioning neuroendocrine tumor of pancreas?
A functioning neuroendocrine tumor of the pancreas (also called a functional pancreatic NET, or F-pNET) is a rare type of tumor that grows in the pancreas — a gland in your belly that helps with digestion and controls blood sugar. What makes these tumors special is that they are 'functioning,' meaning they actively release hormones into your bloodstream. Depending on which hormone is released, the tumor has a specific name — for example, insulinoma (releases insulin), gastrinoma (releases gastrin), glucagonoma, VIPoma, or somatostatinoma. These extra hormones cause a wide range of symptoms tha
At what age does Functioning neuroendocrine tumor of pancreas typically begin?
Typical onset of Functioning neuroendocrine tumor of pancreas is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Functioning neuroendocrine tumor of pancreas?
Yes — 3 recruiting clinical trials are currently listed for Functioning neuroendocrine tumor 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 Functioning neuroendocrine tumor of pancreas?
10 specialists and care centers treating Functioning neuroendocrine tumor of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.