Digestive system neuroendocrine neoplasm
diseaseOn this page
Also known as alimentary part of gastrointestinal system NETalimentary part of gastrointestinal system neuroendocrine neoplasmalimentary part of gastrointestinal system neuroendocrine tumoralimentary part of gastrointestinal system neuroendocrine tumor, well differentiated, low or intermediate gradealimentary part of gastrointestinal system neuroendocrine tumourcarcinoid tumorcarcinoid tumor of digestive systemcarcinoid tumourcarcinoid tumour of digestive systemdigestive system NETdigestive system neuroendocrine tumordigestive system neuroendocrine tumor, well differentiated, low or intermediate gradedigestive system neuroendocrine tumourgastro-enteropancreatic neuroendocrine tumorgastro-enteropancreatic neuroendocrine tumourgastroenteropancreatic endocrine tumorgastroenteropancreatic endocrine tumourgastroenteropancreatic neuroendocrine neoplasmgastrointestinal neuroendocrine neoplasm
Summary
Digestive system neuroendocrine neoplasm (MONDO:0024503) is a cancer (an umbrella term covering 7 Mondo subtypes) and 78 clinical trials. Top therapeutic interventions include lutetium oxodotreotide lu-177, cabozantinib, and edotreotide gallium ga-68. A subtype of digestive system disorder — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Umbrella term: 7 Mondo subtypes
- Clinical trials: 78
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | digestive system neuroendocrine neoplasm |
| Mondo ID | MONDO:0024503 |
| MeSH | C535650 |
| Orphanet | 100092 |
| NCIT | C27721 |
| UMLS | C1333799 |
| MedGen | 232237 |
| GARD | 0002437 |
| Anatomy (UBERON) | UBERON:0005409 |
| Is cancer (heuristic) | yes |
Also known as: alimentary part of gastrointestinal system NET · alimentary part of gastrointestinal system neuroendocrine neoplasm · alimentary part of gastrointestinal system neuroendocrine tumor · alimentary part of gastrointestinal system neuroendocrine tumor, well differentiated, low or intermediate grade · alimentary part of gastrointestinal system neuroendocrine tumour · carcinoid tumor · carcinoid tumor of digestive system · carcinoid tumour · carcinoid tumour of digestive system · digestive system NET · digestive system neuroendocrine neoplasm · digestive system neuroendocrine tumor · digestive system neuroendocrine tumor, well differentiated, low or intermediate grade · digestive system neuroendocrine tumour · gastro-enteropancreatic neuroendocrine tumor · gastro-enteropancreatic neuroendocrine tumour · gastroenteropancreatic endocrine tumor · gastroenteropancreatic endocrine tumour · gastroenteropancreatic neuroendocrine neoplasm · gastrointestinal neuroendocrine neoplasm (+5 more)
Disease family
This is a subtype of digestive system 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 › digestive system neuroendocrine neoplasm
Related subtypes (30): benign digestive system neoplasm, autoimmune disorder of gastrointestinal tract, gastrointestinal mucositis, diarrheal disease, pancreas disorder, hepatobiliary disorder, digestive system cancer, peptic ulcer disease, stomach disorder, intestinal disorder, Meckel diverticulum, Cronkhite-Canada syndrome, diverticulosis, small-intestinal, diverticulosis of bowel, hernia, and retinal detachment, congenital enteropathy due to enteropeptidase deficiency, hereditary mixed polyposis syndrome, caudal duplication, Moyamoya disease with early-onset achalasia, hyperplastic polyposis syndrome, thoraco-abdominal enteric duplication, digestive duplication, juvenile polyposis syndrome, umbilical cord ulceration-intestinal atresia syndrome, growth retardation-mild developmental delay-chronic hepatitis syndrome, common mesentery, neoplasm of oropharynx, gastrointestinal polyp, digestive system infectious disorder, upper digestive tract disorder, congenital peritoneal encapsulation
Subtypes (7): digestive system neuroendocrine tumor, grade 1/2, intestinal neuroendocrine neoplasm, gastric neuroendocrine neoplasm, pancreatic neuroendocrine neoplasm, salivary gland small cell carcinoma, liver neuroendocrine carcinoma, gallbladder neuroendocrine neoplasm
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: 78.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 35 |
| Not specified | 16 |
| PHASE1 | 10 |
| PHASE3 | 8 |
| PHASE1/PHASE2 | 6 |
| PHASE4 | 2 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT06016855 | PHASE4 | RECRUITING | Surgical Debulking Prior to Peptide Receptor Radionuclide Therapy in Well Differentiated Gastroenteropancreatic Neuroendocrine Tumors |
| NCT02779257 | PHASE4 | WITHDRAWN | Pasireotide Treatment for Neuroendocrine Tumor |
| NCT03375320 | PHASE3 | ACTIVE_NOT_RECRUITING | Testing Cabozantinib in Patients With Advanced Pancreatic Neuroendocrine and Carcinoid Tumors |
| NCT03972488 | PHASE3 | ACTIVE_NOT_RECRUITING | Study to Evaluate the Efficacy and Safety of Lutathera in Patients With Grade 2 and Grade 3 Advanced GEP-NET |
| NCT05050942 | PHASE3 | ACTIVE_NOT_RECRUITING | A Trial to Assess Efficacy and Safety of Octreotide Subcutaneous Depot in Patients With GEP-NET |
| NCT05477576 | PHASE3 | RECRUITING | Study of RYZ101 Compared With SOC in Pts w Inoperable SSTR+ Well-differentiated GEP-NET That Has Progressed Following 177Lu-SSA Therapy |
| NCT07087054 | PHASE3 | RECRUITING | Carcinoid Syndrome Efficacy Study Featuring an Oral Daily Paltusotine Regimen |
| NCT00227136 | PHASE3 | TERMINATED | Effect of Oral 5-HTP Intake on Urinary 5-HIAA Excretion |
| NCT00412061 | PHASE3 | COMPLETED | Everolimus and Octreotide in Patients With Advanced Carcinoid Tumor |
| NCT01373736 | PHASE3 | UNKNOWN | 123I-MIBG Scintigraphy in Patients Being Evaluated for Neuroendocrine Tumors |
| NCT02628067 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of Pembrolizumab (MK-3475) in Participants With Advanced Solid Tumors (MK-3475-158/KEYNOTE-158) |
| NCT02754297 | PHASE2 | ACTIVE_NOT_RECRUITING | Personalized PRRT of Neuroendocrine Tumors |
| NCT03600233 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of CVM-1118 for Patients With Advanced Neuroendocrine Tumors |
| NCT03891784 | PHASE2 | ACTIVE_NOT_RECRUITING | Abemaciclib in Treating Patients With Advanced, Refractory, and Unresectable Digestive System Neuroendocrine Tumors |
| NCT03950609 | PHASE2 | ACTIVE_NOT_RECRUITING | Lenvatinib and Everolimus in Treating Patients With Advanced, Unresectable Carcinoid Tumors |
| NCT05263050 | PHASE2 | RECRUITING | Trial of an Alternative Cabozantinib Dosing Schedule in Metastatic Renal Cell Carcinoma and Neuroendocrine Tumors |
| NCT05969860 | PHASE2 | RECRUITING | At-Home Cancer Directed Therapy Versus in Clinic for the Treatment of Patients With Advanced Cancer |
| NCT06202066 | PHASE2 | RECRUITING | Temozolomide and Survivin Long Peptide Vaccine (SurVaxM) for the Treatment of Patients With Progressing Metastatic Neuroendocrine Carcinomas |
| NCT06790706 | PHASE2 | RECRUITING | IMMUNORARE5: A National Platform of 5 Academic Phase II Trials Coordinated by Lyon University Hospital to Assess the Safety and the Efficacy of the IMMUNOtherapy With Domvanalimab + Zimberelimab Combination in Patients With Advanced RARE Cancers |
| NCT00088595 | PHASE2 | COMPLETED | Study Evaluating SOM230 in Patients With Metastatic Carcinoid Tumors |
| NCT00328497 | PHASE2 | COMPLETED | A Combination Study to Determine the Safety and Efficacy of Panzem NCD With Avastin in Metastatic Carcinoid Tumors |
| NCT00780663 | PHASE2 | COMPLETED | Quarfloxin in Patients With Low to Intermediate Grade Neuroendocrine Carcinoma |
| NCT00947167 | PHASE2 | TERMINATED | A Phase II Study of Pertuzumab and Erlotinib for Metastatic or Unresectable Neuroendocrine Tumors |
| NCT01024387 | PHASE2 | COMPLETED | AMG 479 in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors |
| NCT01175096 | PHASE1/PHASE2 | UNKNOWN | Safety and Tolerability Profile of RAD001 Daily in Chinese Patients With Advanced Pulmonary Neuroendocrine Tumor |
| NCT01253161 | PHASE2 | COMPLETED | Study of Pasireotide Long Acting Release (LAR) in Patients With Metastatic Neuroendocrine Tumors (NETs) |
| NCT01435122 | PHASE2 | COMPLETED | A Study of Axitinib in Advanced Carcinoid Tumors |
| NCT01466036 | PHASE2 | COMPLETED | Cabozantinib in Advanced Pancreatic Neuroendocrine and Carcinoid Tumors |
| NCT01619865 | PHASE1/PHASE2 | COMPLETED | Safety of 68Ga-DOTA-tyr3-Octreotide PET in Diagnosis of Solid Tumors |
| NCT01731925 | PHASE2 | UNKNOWN | A Study of Sunitinib Versus Placebo in Combination With Lanreotide in Patients With Progressive Advanced/Metastatic Midgut Carcinoid Tumors |
| NCT01782443 | PHASE2 | COMPLETED | Ziv-Aflibercept for Advanced Progressive Carcinoid Tumors |
| NCT02038738 | PHASE1/PHASE2 | UNKNOWN | 68Ga-DOTATATE PET Scan Imaging in Patients With Neuroendocrine Tumors |
| NCT02177773 | PHASE1/PHASE2 | TERMINATED | GA-68 DOTA-TOC of Somatostatin Positive Malignancies |
| NCT02399215 | PHASE2 | COMPLETED | Nintedanib in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors |
| NCT02420691 | PHASE2 | COMPLETED | Ribociclib in Treating Patients With Advanced Neuroendocrine Tumors of Foregut Origin |
| NCT02441062 | PHASE2 | COMPLETED | Impact of Ga-68 DOTATOC PET-CT Imaging in Management of Neuroendocrine Tumors |
| NCT02575300 | PHASE2 | COMPLETED | Phase II Study of Ibrutinib in Advanced Carcinoid and Pancreatic Neuroendocrine Tumors |
| NCT02795858 | PHASE2 | COMPLETED | A Phase II Study of Ramucirumab With Somatostatin Analog Therapy in Patients With Advanced, Progressive Carcinoid Tumors |
| NCT02859064 | PHASE2 | TERMINATED | Study of Lanreotide in Patients With Metastatic Gastrointestinal Neuroendocrine Tumors Who Are Undergoing Liver-directed Radioembolization With Yttrium-90 Microspheres |
| NCT03042780 | PHASE2 | TERMINATED | FOLFIRINOX in Metastatic High Grade Gastroenteropancreatic Neuroendocrine Carcinomas |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| LUTETIUM OXODOTREOTIDE LU-177 | 4 | 7 |
| CABOZANTINIB | 4 | 5 |
| EDOTREOTIDE GALLIUM GA-68 | 4 | 4 |
| PASIREOTIDE | 4 | 4 |
| LANREOTIDE | 4 | 3 |
| OCTREOTIDE | 4 | 3 |
| TEMOZOLOMIDE | 4 | 2 |
| ABEMACICLIB | 4 | 1 |
| AXITINIB | 4 | 1 |
| DIAZOXIDE | 4 | 1 |
| EVEROLIMUS | 4 | 1 |
| IOBENGUANE I 131 | 4 | 1 |
| NINTEDANIB | 4 | 1 |
| PERTUZUMAB | 4 | 1 |
| PIFLUFOLASTAT F18 | 4 | 1 |
| RAMUCIRUMAB | 4 | 1 |
| RIBOCICLIB | 4 | 1 |
| SUNITINIB | 4 | 1 |
| PALTUSOTINE | 3 | 2 |
| VELIPARIB | 3 | 2 |
| ACTINIUM AC 225 DOTATATE | 3 | 1 |
| DOMVANALIMAB | 3 | 1 |
| SOMATOSTATIN | 3 | 1 |
| TRIAPINE | 3 | 1 |
| ZIMBERELIMAB | 3 | 1 |
| FOSLINANIB | 2 | 1 |
| LUTETIUM LU177 EDOTREOTIDE | 2 | 1 |
| QUARFLOXIN | 2 | 1 |
| SATOREOTIDE TRIZOXETAN | 2 | 1 |
| SVN53-67/M57-KLH PEPTIDE VACCINE | 2 | 1 |
Related Atlas pages
- Drugs: LUTETIUM OXODOTREOTIDE LU-177, Cabozantinib, EDOTREOTIDE GALLIUM GA-68, Pasireotide, Lanreotide, Octreotide, Temozolomide, Abemaciclib, Axitinib, Diazoxide, Everolimus, IOBENGUANE I 131, Nintedanib, Pertuzumab, PIFLUFOLASTAT F18, Ramucirumab, Ribociclib, Sunitinib, Paltusotine, Veliparib, ACTINIUM AC 225 DOTATATE, Domvanalimab, Somatostatin, Triapine, Zimberelimab