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Pancreatic endocrine tumor, also known as pancreatic neuroendocrine tumor (PNET), describes a group of endocrine tumors originating in the pancreas that are usually indolent and benign, but may have the potential to be malignant. They can be functional, exhibiting a hormonal hypersecretion syndrome, but can be non-functional presenting with non-specific symptoms and include insulinoma, glucagonoma, VIPoma, somatostatinoma (SSoma), PPoma and Zollinger-Ellison syndrome (ZES, or gastrinoma) and other ectopic hormone producing tumors (such as GRFoma).
Biomarker and diagnostic research for pancreatic neuroendocrine tumor has been reported in the published literature.
Data assembled from 5 of 12 sources · Last updated Sep 20, 2026, 4:50 AM UTC
European rare disease database
Genetic and Rare Diseases Info Center
No approved treatments are currently available for pancreatic neuroendocrine tumor. An additional 1 compound holds orphan drug designation.
While no drugs are FDA-approved specifically for pancreatic neuroendocrine tumor, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for pancreatic neuroendocrine tumor. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor | Designated | Exclusivity End | Designation Status |
|---|---|---|---|---|---|
Foslinanib | Foslinanib | TaiRx, Inc. | 2025 | — | Designated |
Gene therapy approaches for pancreatic neuroendocrine tumor have been reported in the published literature.
42 trials found
Estimated prevalence: 1-5 in 10,000 (Uncommon).
42 clinical trials registered, 8 recruiting. Interventions under study include drug therapy, other interventions, procedural interventions, and biologic therapy. Pipeline includes 1 PHASE4, 3 PHASE3, 14 PHASE2. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT00830557](https://clinicaltrials.gov/study/NCT00830557) | Collecting Medical Information and Tissue Samples From Patients With Pancreatic Cancer or Other Pancreatic Disorders | — | Mayo Clinic | RECRUITING |
[NCT04119024](https://clinicaltrials.gov/study/NCT04119024) | Gene Modified Immune Cells After Conditioning Regimen for the Treatment of Stage IIIC or IV Melanoma or Metastatic Solid Tumors | PHASE1 | Anusha Kalbasi | RECRUITING |
[NCT06523582](https://clinicaltrials.gov/study/NCT06523582) | Genetic Bases of Neuroendocrine Neoplasms in Mexican Patients | — | Universidad Nacional Autonoma de Mexico | RECRUITING |
[NCT07439757](https://clinicaltrials.gov/study/NCT07439757) | AI-Powered Precision Decision-Making for Pancreatic Diseases | — | Changhai Hospital | RECRUITING |
[NCT06592989](https://clinicaltrials.gov/study/NCT06592989) | A Clinical Study of Sorafenib Combined With Gefitinib for the Treatment of pNET | — | Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine | RECRUITING |
294 publications have been identified in PubMed for pancreatic neuroendocrine tumor. Kisho has analyzed 154 by research type. Research spans Case Report / Case Series (21%), Basic Science / Preclinical (19%), and Diagnostic / Biomarker (18%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 33 | 21% |
Laboratory research | 29 | 19% |
Testing and diagnosis research | 28 | 18% |
Research summaries | 24 | 16% |
Clinical study results | 19 | 12% |
Disease patterns and progression | 13 |
Aliberti G (2026). [PMID: 41633869](https://pubmed.ncbi.nlm.nih.gov/41633869/). *Diagn Interv Imaging*. [Other]
Xu L (2026). [PMID: 42160318](https://pubmed.ncbi.nlm.nih.gov/42160318/). *Endocr Relat Cancer*. [Basic Science / Preclinical]
Singhi AD (2026). [PMID: 41388172](https://pubmed.ncbi.nlm.nih.gov/41388172/). *Ann Surg Oncol*. [Clinical Trial Publication]
Cai Z (2026). [PMID: 41591671](https://pubmed.ncbi.nlm.nih.gov/41591671/). *Discov Oncol*. [Basic Science / Preclinical]
Choi GW (2026). [PMID: 41680035](https://pubmed.ncbi.nlm.nih.gov/41680035/). *Pancreatology*. [Diagnostic / Biomarker]
Expert Panel on Gastrointestinal Imaging (2026). [PMID: 41823937](https://pubmed.ncbi.nlm.nih.gov/41823937/). *J Am Coll Radiol*. [Review / Meta-Analysis]
Kjær MB (2026). [PMID: 41761637](https://pubmed.ncbi.nlm.nih.gov/41761637/). *J Neuroendocrinol*. [Basic Science / Preclinical]
Zhang L (2026). [PMID: 41642264](https://pubmed.ncbi.nlm.nih.gov/41642264/). *J Am Coll Surg*. [Epidemiology / Natural History]
Lamback E (2026). [PMID: 42007244](https://pubmed.ncbi.nlm.nih.gov/42007244/). *JCEM Case Rep*. [Case Report / Case Series]
Watanabe H (2026). [PMID: 41051970](https://pubmed.ncbi.nlm.nih.gov/41051970/). *Int J Surg Pathol*. [Review / Meta-Analysis]
New treatment approaches | 7 | 5% |
Other research | 1 | 1% |
AI-curated news mentioning pancreatic neuroendocrine tumor
Updated Jun 15, 2026
A study published in PubMed explores the use of robotic subtotal pancreatectomy for treating multifocal neuroendocrine tumors of the pancreas. This surgical approach may enhance recovery and reduce complications compared to traditional methods.
Recent research highlights pancreatic neuroendocrine tumors as part of the tumor spectrum associated with Lynch syndrome, indicating a broader impact of mismatch repair deficiency. This discovery could influence future screening and treatment strategies for affected patients.
A recent case report details the occurrence of metachronous primary pancreatic neuroendocrine tumor alongside adenocarcinoma. This study contributes to the understanding of pancreatic tumors and their potential interactions.