Functional pancreatic neuroendocrine tumor
diseaseOn this page
Also known as functional pancreatic NETfunctioning neuroendocrine tumour of pancreasfunctioning pancreatic endocrine tumorfunctioning pancreatic endocrine tumourfunctioning pancreatic NETfunctioning pancreatic neuroendocrine tumorfunctioning pancreatic neuroendocrine tumourfunctioning PNETfunctioning well differentiated pancreatic endocrine neoplasmfunctioning well differentiated pancreatic endocrine tumorfunctioning well differentiated pancreatic endocrine tumourfunctioning well-differentiated NEN of pancreasfunctioning well-differentiated neuroendocrine neoplasm of pancreasfunctioning well-differentiated pancreatic NENfunctioning well-differentiated pancreatic neuroendocrine neoplasmsyndromic pancreatic NETsyndromic pancreatic neuroendocrine tumorsyndromic pancreatic neuroendocrine tumour
Summary
Functional pancreatic neuroendocrine tumor (MONDO:0023206) is a cancer and 3 clinical trials. Top therapeutic interventions include cabozantinib, ribociclib, and veliparib. A subtype of pancreatic neuroendocrine tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 3
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | functional pancreatic neuroendocrine tumor |
| Mondo ID | MONDO:0023206 |
| Orphanet | 506060 |
| NCIT | C45840 |
| UMLS | C1708107 |
| MedGen | 310778 |
| GARD | 0022053 |
| Is cancer (heuristic) | yes |
Also known as: functional pancreatic NET · functional pancreatic neuroendocrine tumor · functioning neuroendocrine tumour of pancreas · functioning pancreatic endocrine tumor · functioning pancreatic endocrine tumour · functioning pancreatic NET · functioning pancreatic neuroendocrine tumor · functioning pancreatic neuroendocrine tumour · functioning PNET · functioning well differentiated pancreatic endocrine neoplasm · functioning well differentiated pancreatic endocrine tumor · functioning well differentiated pancreatic endocrine tumour · functioning well-differentiated NEN of pancreas · functioning well-differentiated neuroendocrine neoplasm of pancreas · functioning well-differentiated pancreatic NEN · functioning well-differentiated pancreatic neuroendocrine neoplasm · syndromic pancreatic NET · syndromic pancreatic neuroendocrine tumor · syndromic pancreatic neuroendocrine tumour
Disease family
This is a subtype of pancreatic neuroendocrine tumor. 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 › digestive system neuroendocrine neoplasm › digestive system neuroendocrine tumor, grade 1/2 › pancreatic neuroendocrine tumor › functional pancreatic neuroendocrine tumor
Related subtypes (10): pancreatic delta cell neuroendocrine tumor, pancreatic gastrin-producing neuroendocrine tumor, non-functional pancreatic neuroendocrine tumor, pancreatic insulin-producing neuroendocrine tumor, somatostatinoma, GRFoma, PPoma, glucagonoma, VIPoma, pancreatic neuroendocrine tumor G1
Subtypes (3): pancreatic ACTH-producing neuroendocrine tumor, pancreatic gastrinoma, pancreatic insulinoma
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
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 3.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE3 | 1 |
| PHASE2 | 1 |
| PHASE1 | 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 |
| NCT02420691 | PHASE2 | COMPLETED | Ribociclib in Treating Patients With Advanced Neuroendocrine Tumors of Foregut Origin |
| NCT02831179 | PHASE1 | WITHDRAWN | Veliparib, Capecitabine, and Temozolomide in Patients With Advanced, Metastatic, and Recurrent Neuroendocrine Tumor |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CABOZANTINIB | 4 | 3 |
| RIBOCICLIB | 4 | 1 |
| VELIPARIB | 3 | 2 |
Related Atlas pages
- Drugs: Cabozantinib, Ribociclib, Veliparib