Pancreatic neuroendocrine neoplasm
diseaseOn this page
Also known as endocrine pancreas cancerendocrine pancreas neoplasmendocrine pancreas neoplasm (disease)endocrine pancreas tumorendocrine pancreas tumourislet cell neoplasmislet cell tumorIslet cell tumorsislet cell tumourIslet cell tumoursIslet of Langerhans tumorIslet of Langerhans tumourmalignant pancreatic endocrine tumormalignant pancreatic endocrine tumourneoplasm of endocrine pancreaspancreatic endocrine neoplasmpancreatic NENPNENtumor of endocrine pancreas
Summary
Pancreatic neuroendocrine neoplasm (MONDO:0005815) is a cancer and 23 clinical trials. Top therapeutic interventions include cabozantinib, octreotide, and bevacizumab. A subtype of endocrine pancreas disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 23
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | pancreatic neuroendocrine neoplasm |
| Mondo ID | MONDO:0005815 |
| EFO | EFO:0007331 |
| Orphanet | 506052 |
| DOID | DOID:1799 |
| ICD-11 | 1421495979 |
| NCIT | C27031 |
| SNOMED CT | 254611009 |
| UMLS | C5848399 |
| MedGen | 1848937 |
| GARD | 0022052 |
| Anatomy (UBERON) | UBERON:0000016 |
| Is cancer (heuristic) | yes |
Also known as: endocrine pancreas cancer · endocrine pancreas neoplasm · endocrine pancreas neoplasm (disease) · endocrine pancreas tumor · endocrine pancreas tumour · islet cell neoplasm · islet cell tumor · Islet cell tumors · islet cell tumour · Islet cell tumours · Islet of Langerhans tumor · Islet of Langerhans tumour · malignant pancreatic endocrine tumor · malignant pancreatic endocrine tumour · neoplasm of endocrine pancreas · pancreatic endocrine neoplasm · pancreatic NEN · pancreatic neuroendocrine neoplasm · PNEN · tumor of endocrine pancreas (+1 more)
Disease family
This is a subtype of endocrine pancreas disorder. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by body system or component › digestive system disorder › pancreas disorder › endocrine pancreas disorder › pancreatic neuroendocrine neoplasm
Related subtypes (11): gastrin secretion abnormality, abnormality of glucagon secretion, hyperinsulinism, post-surgical hypoinsulinemia, pancreatic cholera, diabetes mellitus, aggressive insulitis, benign insulitis, islet cell adenomatosis, insulin-resistance syndrome type A, insulin-resistance syndrome type B
Subtypes (2): pancreatic endocrine carcinoma, pancreatic neuroendocrine tumor
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
Drugs indicated for this disease
No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Everolimus, Interferon, Melphalan, Nimotuzumab, Octreotide, Pasireotide.
Clinical trials & evidence
Clinical trials
Clinical trials: 23.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 11 |
| Not specified | 6 |
| PHASE1 | 3 |
| PHASE2/PHASE3 | 1 |
| PHASE3 | 1 |
| PHASE1/PHASE2 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03375320 | PHASE3 | ACTIVE_NOT_RECRUITING | Testing Cabozantinib in Patients With Advanced Pancreatic Neuroendocrine and Carcinoid Tumors |
| NCT00227617 | PHASE2/PHASE3 | TERMINATED | Combination Chemotherapy and Bevacizumab in Treating Patients With Advanced Neuroendocrine Tumors |
| NCT05316480 | PHASE2 | NOT_YET_RECRUITING | Nimotuzumab in EGFR Highly Expressed Pancreatic Neuroendocrine Neoplasms |
| NCT06202066 | PHASE2 | RECRUITING | Temozolomide and Survivin Long Peptide Vaccine (SurVaxM) for the Treatment of Patients With Progressing Metastatic Neuroendocrine Carcinomas |
| NCT00017199 | PHASE2 | COMPLETED | PS-341 in Treating Patients With Metastatic Neuroendocrine Tumors |
| NCT00019786 | PHASE2 | COMPLETED | Isolated Hepatic Perfusion With Melphalan in Treating Patients With Primary Unresectable Liver Cancer or Liver Metastases |
| NCT00027638 | PHASE2 | COMPLETED | Thalidomide in Treating Patients With Metastatic Neuroendocrine Tumors |
| NCT00416767 | PHASE2 | COMPLETED | Combination Chemotherapy as First-Line Therapy in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors of the Duodenum or Pancreas That Cannot Be Removed By Surgery |
| NCT00427349 | PHASE2 | COMPLETED | AMG 706 and Octreotide in Treating Patients With Low-Grade Neuroendocrine Tumors |
| NCT00434109 | PHASE2 | COMPLETED | Phase II Study of Sunitinib Malate Following Hepatic Artery Embolization |
| NCT00466856 | PHASE2 | TERMINATED | Internal Radiation Therapy in Treating Patients With Liver Metastases From Neuroendocrine Tumors |
| NCT00602082 | PHASE2 | COMPLETED | Capecitabine and Streptozocin With or Without Cisplatin in Treating Patients With Unresectable or Metastatic Neuroendocrine Tumors |
| NCT01374451 | PHASE2 | TERMINATED | Efficacy of Everolimus Alone or in Combination With Pasireotide LAR in Advanced PNET |
| NCT02038738 | PHASE1/PHASE2 | UNKNOWN | 68Ga-DOTATATE PET Scan Imaging in Patients With Neuroendocrine Tumors |
| NCT00002947 | PHASE1 | TERMINATED | Indium In 111 Pentetreotide in Treating Patients With Refractory Cancer |
| NCT00006368 | PHASE1 | COMPLETED | Yttrium Y 90 SMT 487 in Treating Patients With Refractory or Recurrent Cancer |
| NCT00049023 | PHASE1 | COMPLETED | Radiolabeled Octreotide in Treating Children With Advanced or Refractory Solid Tumors |
| NCT00830557 | Not specified | RECRUITING | Collecting Medical Information and Tissue Samples From Patients With Pancreatic Cancer or Other Pancreatic Disorders |
| NCT05746182 | Not specified | RECRUITING | Genetic Predisposition Testing Program for Pancreatic Neuroendocrine Neoplasms |
| NCT06592989 | Not specified | RECRUITING | A Clinical Study of Sorafenib Combined With Gefitinib for the Treatment of pNET |
| NCT00454376 | Not specified | UNKNOWN | Disease-Specific Questionnaire in Assessing Quality of Life in Patients With Gastrointestinal-Related Neuroendocrine Tumors |
| NCT00730483 | Not specified | TERMINATED | Doxorubicin Beads in Treating Patients With Unresectable Liver Metastases From Neuroendocrine Tumors |
| NCT02400996 | Not specified | COMPLETED | An Analysis of Presentation and Outcome Following Treatment of Pancreatic Endocrine Neoplasms |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CABOZANTINIB | 4 | 3 |
| OCTREOTIDE | 4 | 2 |
| BEVACIZUMAB | 4 | 1 |
| INDIUM IN 111 PENTETREOTIDE | 4 | 1 |
| LEUCOVORIN | 4 | 1 |
| OXALIPLATIN | 4 | 1 |
| STREPTOZOCIN | 4 | 1 |
| SUNITINIB MALATE | 4 | 1 |
| NIMOTUZUMAB | 3 | 1 |
| EDOTREOTIDE YTTRIUM Y-90 | 2 | 2 |
| SVN53-67/M57-KLH PEPTIDE VACCINE | 2 | 1 |
| CHEMBL4854670 | 0 | 1 |
| CHEMBL3350037 | 0 | 1 |
| CHEMBL3109278 | 0 | 1 |
| CHEMBL4783128 | 0 | 1 |
| S-ROLIPRAM | 0 | 1 |