Overview
A serotonin-producing neuroendocrine tumor of the pancreas (also called a pancreatic serotonin-secreting NET, or pancreatic carcinoid tumor) is a rare type of tumor that grows in the pancreas. Unlike most pancreatic cancers, this tumor is made up of specialized cells called neuroendocrine cells, which normally help control digestion and hormone release. What makes this tumor unusual is that it releases large amounts of serotonin — a chemical messenger — directly into the bloodstream. This can cause a wide range of symptoms throughout the body. Because serotonin affects many organs, people with this tumor often experience flushing of the skin, diarrhea, stomach cramps, and wheezing. Over time, the excess serotonin can damage the heart valves, a condition called carcinoid heart disease. The tumor may also grow slowly and spread to the liver or other organs before it is discovered, since early symptoms can be easy to miss or mistake for other conditions. Treatment depends on how far the tumor has spread. Surgery to remove the tumor is the main goal when possible. Medications called somatostatin analogs (such as octreotide and lanreotide) can help control symptoms and slow tumor growth. Newer targeted therapies like everolimus and sunitinib, as well as a radioactive treatment called PRRT (peptide receptor radionuclide therapy), are also available. With the right care, many people live for years after diagnosis.
Also known as:
Key symptoms:
Sudden redness or flushing of the face and neckFrequent watery diarrheaStomach cramping or painWheezing or shortness of breathRapid or irregular heartbeatFatigue and low energyWeight loss without tryingSwelling in the legs or anklesNausea or vomitingLow blood sugar episodes (dizziness, shakiness, sweating)
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
3 eventsShanghai General Hospital, Shanghai Jiao Tong University School of Medicine
University of California, San Francisco
Fudan University — PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
2 availableNETSPOT
indicated for use with positron emission tomography (PET) for localization of somatostatin receptor positive neuroendocrine tumors (NETs)
Detectnet
indicated for use with positron emission tomography (PET) for localization of somatostatin receptor positive neuroendocrine tumors (NETs) in adult patients
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 Serotonin-producing neuroendocrine tumor of pancreas.
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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.Has my tumor spread, and if so, where has it gone?,Is surgery an option for me, and what are the risks?,Should I start a somatostatin analog injection, and how often will I need it?,Do I need to be tested for an inherited syndrome like MEN1 or von Hippel-Lindau?,How will you monitor my heart for carcinoid heart disease?,Am I a candidate for PRRT (Lutathera) treatment?,Are there any clinical trials I should consider?
Common questions about Serotonin-producing neuroendocrine tumor of pancreas
What is Serotonin-producing neuroendocrine tumor of pancreas?
A serotonin-producing neuroendocrine tumor of the pancreas (also called a pancreatic serotonin-secreting NET, or pancreatic carcinoid tumor) is a rare type of tumor that grows in the pancreas. Unlike most pancreatic cancers, this tumor is made up of specialized cells called neuroendocrine cells, which normally help control digestion and hormone release. What makes this tumor unusual is that it releases large amounts of serotonin — a chemical messenger — directly into the bloodstream. This can cause a wide range of symptoms throughout the body. Because serotonin affects many organs, people wit
How is Serotonin-producing neuroendocrine tumor of pancreas inherited?
Serotonin-producing neuroendocrine tumor of pancreas follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Serotonin-producing neuroendocrine tumor of pancreas typically begin?
Typical onset of Serotonin-producing neuroendocrine tumor of pancreas is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Serotonin-producing neuroendocrine tumor of pancreas?
Yes — 2 recruiting clinical trials are currently listed for Serotonin-producing 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 Serotonin-producing neuroendocrine tumor of pancreas?
1 specialists and care centers treating Serotonin-producing neuroendocrine tumor of pancreas are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.