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Disease

Pancreatic neuroendocrine tumor

Late-stage therapeutic development
Also known as islet cell tumor, islet cell tumors - pancreas, islet cell tumors of the pancreas, islet cell tumour+15 more

islet cell tumor, islet cell tumors - pancreas, islet cell tumors of the pancreas, islet cell tumour, islet cell tumours - pancreas, islet cell tumours of the pancreas, pancreatic NET, well differentiated pancreatic endocrine neoplasm, well differentiated pancreatic endocrine tumor, well differentiated pancreatic endocrine tumour, well-differentiated NEN of pancreas, well-differentiated neuroendocrine neoplasm of pancreas, well-differentiated pancreatic NEN, well-differentiated pancreatic neuroendocrine neoplasm, PANET, neuroendocrine tumor of pancreas, neuroendocrine tumour of pancreas, pancreatic endocrine tumor, pancreatic endocrine tumour.

17
Clinical trials
5
Associated genes
1
Related proteins

What's happening now

An analyst briefing on current research, clinical, regulatory and industry activity surrounding this disease.

Executive briefingUpdating summary…Momentum: Moderate
Key developments
  • 3 clinical trials expected to report results, the earliest in Q2 2027.
Upcoming Milestones
Clinical Milestones6View
Results posted 1
Results expected 3
Recently completed 1
Other updates 1
Activity timeline6

Therapeutic landscape

Therapies with a regulatory footing for this condition, alongside the wider set of agents co-studied with it in the literature.

Approved & established therapies
Belzutifanapproved

Approval — Renal cell carcinoma (RCC) Welireg is indicated as monotherapy for the treatment of adul… (2025)

Sunitinibapproved

Approval — Gastrointestinal stromal tumour (GIST) Sunitinib Accord is indicated for the treatment of… (2021)

Cabozantinibapproved

Accelerated approval — Renal Cell Carcinoma (RCC) Cabometyx is indicated as monotherapy for advanced renal cell… (2016)

Everolimusapproved

Approval — Hormone-receptor-positive advanced breast cancer Afinitor is indicated for the treatment… (2009)

Clinical trials

13 sponsors · 0 new · 0 completed in the last 12 months (net -1)

The current development programme across all trial phases.

Clinical programme
17
All trials
6
Active
4
Late-stage
6
Completed
Late-stage studies

A Phase 2/3, Multicenter, Randomized Open-Label Study of Zanzalintinib vs Everolimus in Participants With Previously Treated, Unresectable, Locally Advanced or Metastatic Neuroendocrine Tumors

Phase 2/3 · Recruiting · Exelixis

Surgical Debulking Prior to Peptide Receptor Radionuclide Therapy (PRRT) in Patients With Well Differentiated Gastroenteropancreatic Neuroendocrine Tumors

Phase 4 · Recruiting · Vanderbilt-Ingram Cancer Center

Randomized, Double-Blinded Phase III Study of CABozantinib Versus Placebo IN Patients With Advanced NEuroendocrine Tumors After Progression on Prior Therapy (CABINET)

Phase 3 · Active, not recruiting · National Cancer Institute (NCI)

A SINGLE-ARM OPEN-LABEL INTERNATIONAL MULTI-CENTER STUDY OF THE EFFICACY AND SAFETY OF SUNITINIB MALATE (SU011248, SUTENT (REGISTERED)) IN PATIENTS WITH PROGRESSIVE ADVANCED METASTATIC WELL-DIFFERENTIATED UNRESECTABLE PANCREATIC NEUROENDOCRINE TUMORS

Phase 4 · Completed · Pfizer
Recruiting

A Phase 2/3, Multicenter, Randomized Open-Label Study of Zanzalintinib vs Everolimus in Participants With Previously Treated, Unresectable, Locally Advanced or Metastatic Neuroendocrine Tumors

Phase 2/3 · Recruiting · Exelixis

Surgical Debulking Prior to Peptide Receptor Radionuclide Therapy (PRRT) in Patients With Well Differentiated Gastroenteropancreatic Neuroendocrine Tumors

Phase 4 · Recruiting · Vanderbilt-Ingram Cancer Center

A Phase 2 Study to Evaluate the Efficacy and Safety of Belzutifan (MK-6482, Formerly PT2977) Monotherapy in Participants With Advanced Pheochromocytoma/Paraganglioma (PPGL), Pancreatic Neuroendocrine Tumor (pNET), Von Hippel-Lindau (VHL) Disease-Associated Tumors, Advanced Gastrointestinal Stromal Tumor (wt GIST), or Advanced Solid Tumors With HIF-2α Related Genetic Alterations

Phase 2 · Recruiting · Merck Sharp & Dohme LLC
Recently completed

A Phase II Study of MLN0128 (TAK-228) in Rapalog-Resistant Advanced Pancreatic Neuroendocrine Tumors (PNET)

Phase 2 · Completed · National Cancer Institute (NCI)

Phase I/II Study of RAD001 in Combination With Temozolomide in Patients With Advanced Pancreatic Neuroendocrine Tumors

Phase 1/2 · Completed · Dana-Farber Cancer Institute

Temozolomide or Dacarbazine-based Chemotherapy Plus Endostatin in Advanced Pancreatic Neuroendocrine Tumor

Phase 2 · Completed · Peking Union Medical College Hospital

A SINGLE-ARM OPEN-LABEL INTERNATIONAL MULTI-CENTER STUDY OF THE EFFICACY AND SAFETY OF SUNITINIB MALATE (SU011248, SUTENT (REGISTERED)) IN PATIENTS WITH PROGRESSIVE ADVANCED METASTATIC WELL-DIFFERENTIATED UNRESECTABLE PANCREATIC NEUROENDOCRINE TUMORS

Phase 4 · Completed · Pfizer

A Multi-Institutional, Phase II Open-Label Study of AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors

Phase 2 · Completed · Dana-Farber Cancer Institute

Randomized Phase II Study of Everolimus Alone Versus Everolimus Plus Bevacizumab in Patients With Locally Advanced or Metastatic Pancreatic Neuroendocrine Tumors

Phase 2 · Completed · National Cancer Institute (NCI)

Regulatory timeline

Drug regulatory events matched to this condition by indication — EMA.

First approvals
2025emaApproval — Belzutifan· Renal cell carcinoma (RCC) Welireg is indicated as monotherapy for the treatment of adult patients with advanced clear cell renal cell carcinoma that progressed following two or more lines of therapy that included a PD-(L)1 inhibitor and at least two VEGF-targeted therapies. von Hippel-Lindau (VHL) disease-associated tumours Welireg is indicated as monotherapy for the treatment of adult patients with von Hippel-Lindau disease who require therapy for associated, localised renal cell carcinoma (RCC), central nervous system (CNS) haemangioblastomas, or pancreatic neuroendocrine tumours (pNET), and for whom localised procedures are unsuitable. source ↗
2021emaApproval — Sunitinib· Gastrointestinal stromal tumour (GIST) Sunitinib Accord is indicated for the treatment of unresectable and/or metastatic malignant gastrointestinal stromal tumour (GIST) in adults after failure of imatinib treatment due to resistance or intolerance. Metastatic renal cell carcinoma (MRCC) Sunitinib Accord is indicated for the treatment of advanced/metastatic renal cell carcinoma (MRCC) in adults. Pancreatic neuroendocrine tumours (pNET) Sunitinib Accord is indicated for the treatment of unresectable or metastatic, well-differentiated pancreatic neuroendocrine tumours (pNET) with disease progression in adults. source ↗
2016emaAccelerated approval — Cabozantinib· Renal Cell Carcinoma (RCC) Cabometyx is indicated as monotherapy for advanced renal cell carcinoma as first-line treatment of adult patients with intermediate or poor risk, in adults following prior vascular endothelial growth factor (VEGF)-targeted therapy. Cabometyx, in combination with nivolumab, is indicated for the first-line treatment of advanced renal cell carcinoma in adults. Hepatocellular carcinoma (HCC)Cabometyx is indicated as monotherapy for the treatment of hepatocellular carcinoma (HCC) in adults who have previously been treated with sorafenib.Differentiated thyroid carcinoma (DTC)Cabometyx is indicated as monotherapy for the treatment of adult patients with locally advanced or metastatic differentiated thyroid carcinoma (DTC), refractory or not eligible to radioactive iodine (RAI) who have progressed during or after prior systemic therapy.Neuroendocrine Tumours (NET)Cabometyx is indicated for the treatment of adult patients with unresectable or metastatic, well differentiated extra-pancreatic (epNET) and pancreatic (pNET) neuroendocrine tumours who have progressed following at least one prior systemic therapy other than somatostatin analogues. source ↗
2009emaApproval — Everolimus· Hormone-receptor-positive advanced breast cancer Afinitor is indicated for the treatment of hormone-receptor-positive, HER2/neu-negative advanced breast cancer, in combination with exemestane, in post-menopausal women without symptomatic visceral disease after recurrence or progression following a non-steroidal aromatase inhibitor. Neuroendocrine tumours of pancreatic origin Afinitor is indicated for the treatment of unresectable or metastatic, well or moderately differentiated neuroendocrine tumours of pancreatic origin in adults with progressive disease. Neuroendocrine tumours of gastrointestinal or lung origin Afinitor is indicated for the treatment of unresectable or metastatic, well-differentiated (Grade 1 or Grade 2) non-functional neuroendocrine tumours of gastrointestinal or lung origin in adults with progressive disease. Renal-cell carcinoma Afinitor is indicated for the treatment of patients with advanced renal-cell carcinoma, whose disease has progressed on or after treatment with VEGF-targeted therapy. source ↗
2006emaApproval — Sunitinib· Gastrointestinal stromal tumour (GIST) Sutent is indicated for the treatment of unresectable and/or metastatic malignant gastrointestinal stromal tumour (GIST) in adults after failure of imatinib mesilate treatment due to resistance or intolerance. Metastatic renal cell carcinoma (MRCC) Sutent is indicated for the treatment of advanced/metastatic renal cell carcinoma (MRCC) in adults. Pancreatic neuroendocrine tumours (pNET) Sutent is indicated for the treatment of unresectable or metastatic, well-differentiated pancreatic neuroendocrine tumours with disease progression in adults. Experience with Sutent as first-line treatment is limited (see section 5.1). source ↗

European Medicines Agency (CC BY 4.0). Events are matched to this condition by drug indication text — approvals/updates for drugs indicated for it, not disease-specific acts.

Associated genes

5 matches

Genes associated with this disease in the canonical knowledge graph (Open Targets evidence). Number shows the association score.

DAXX0.47EPAS10.44GPC30.37WWTR10.37RABEP10.18

Disease biology

1 match

Proteins whose encoding gene is associated with this disease, reached through the canonical gene→disease graph. Number shows the gene's association score.

Endothelial PAS domain-containing protein 1via EPAS10.44

Reference

Authoritative identity, definition & identifiers.

Synonyms

islet cell tumor, islet cell tumors - pancreas, islet cell tumors of the pancreas, islet cell tumour, islet cell tumours - pancreas, islet cell tumours of the pancreas, pancreatic NET, well differentiated pancreatic endocrine neoplasm, well differentiated pancreatic endocrine tumor, well differentiated pancreatic endocrine tumour, well-differentiated NEN of pancreas, well-differentiated neuroendocrine neoplasm of pancreas, well-differentiated pancreatic NEN, well-differentiated pancreatic neuroendocrine neoplasm, PANET, neuroendocrine tumor of pancreas, neuroendocrine tumour of pancreas, pancreatic endocrine tumor, pancreatic endocrine tumour

References & data sources
  • Disease identity & definition — NLM Medical Subject Headings (MeSH), public domain
  • Clinical trials — ClinicalTrials.gov (U.S. National Library of Medicine)
  • Related entities are derived from literature co-mention (studied together) — associative, not causal.