Lung neuroendocrine neoplasm
diseaseOn this page
Also known as lung NETlung neuroendocrine tumorlung neuroendocrine tumor, well differentiated, low or intermediate gradelung neuroendocrine tumourneuroendocrine neoplasm of lungneuroendocrine neoplasm of the lungpulmonary neuroendocrine neoplasmpulmonary neuroendocrine tumour
Summary
Lung neuroendocrine neoplasm (MONDO:0005454) is a cancer (an umbrella term covering 5 Mondo subtypes) with 1 GWAS associations across 1 studies and 18 clinical trials. Top therapeutic interventions include cabozantinib, fludeoxyglucose f 18, and lutetium oxodotreotide lu-177. A subtype of neuroendocrine neoplasm — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Umbrella term: 5 Mondo subtypes
- GWAS associations: 1
- Clinical trials: 18
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | lung neuroendocrine neoplasm |
| Mondo ID | MONDO:0005454 |
| EFO | EFO:0005220 |
| DOID | DOID:5410 |
| NCIT | C5670 |
| SNOMED CT | 707594002 |
| UMLS | C1334452 |
| MedGen | 277303 |
| GARD | 0024190 |
| Anatomy (UBERON) | UBERON:0002048 |
| Is cancer (heuristic) | yes |
Also known as: lung NET · lung neuroendocrine neoplasm · lung neuroendocrine tumor · lung neuroendocrine tumor, well differentiated, low or intermediate grade · lung neuroendocrine tumour · neuroendocrine neoplasm of lung · neuroendocrine neoplasm of the lung · pulmonary neuroendocrine neoplasm · pulmonary neuroendocrine tumour
Data availability: 1 GWAS association (1 study).
Disease family
This is a subtype of neuroendocrine neoplasm. 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 etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › endocrine gland neoplasm › neuroendocrine neoplasm › lung neuroendocrine neoplasm
Related subtypes (13): paraganglioma, neuroendocrine carcinoma, prostate neuroendocrine neoplasm, ovarian neuroendocrine neoplasm, breast neuroendocrine neoplasm, carcinoid tumor, laryngeal neuroendocrine neoplasm, middle ear neuroendocrine tumor, hereditary pheochromocytoma-paraganglioma, bronchial endocrine tumor, thymic neuroendocrine tumor, uterine corpus neuroendocrine neoplasm, digestive system neuroendocrine neoplasm
Subtypes (5): pulmonary large cell neuroendocrine carcinoma, lung mixed small cell and squamous cell carcinoma, lung carcinoid tumor, combined lung carcinoma, small cell lung carcinoma
Genetics & variants
GWAS landscape
1 GWAS associations across 1 studies. Top hits map to 0 distinct genes (as reported by GWAS).
Top associations by p-value
| rsID | p-value | Gene | Risk allele | Odds ratio |
|---|---|---|---|---|
| rs10827565 | 1e-11 | LINC02630 - MTND5P17 | ? | 2.99 |
Top studies (by case count)
| Study | Lead author | Year | Cases | Controls | Title |
|---|---|---|---|---|---|
| GCST003787 | Du Y | 2016 | 128 | 4,542 | Genetic associations with neuroendocrine tumor risk: results from a genome-wide association study. |
Variant details and genetic-evidence tiers
Tier distribution (top 50 variants)
| Tier | Variants |
|---|---|
| Tier 1: coding | 0 |
| Tier 2: splice/UTR | 0 |
| Tier 3: regulatory | 0 |
| Tier 4: intronic/intergenic | 1 |
MAF distribution
| Bucket | Variants |
|---|---|
| common (>=0.05) | 1 |
| low_freq (0.01-0.05) | 0 |
| rare (<0.01) | 0 |
| unknown | 0 |
Functional consequences
| Consequence | Count |
|---|---|
| intron_variant | 1 |
Top variants
| rsID | Chr | Pos | Alleles | MAF | Consequence | Gene | p-value | Tier |
|---|---|---|---|---|---|---|---|---|
| rs10827565 | 10 | 35955481 | C>G | 0.05 | intron_variant | LINC02630 - MTND5P17 | 1e-11 | Tier 4: intronic/intergenic |
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
1 approved. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Everolimus | Approved (phase 4) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Cabozantinib, Durvalumab, Ribociclib, Temozolomide, Tremelimumab.
Clinical trials & evidence
Clinical trials
Clinical trials: 18.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 8 |
| Not specified | 6 |
| PHASE3 | 2 |
| PHASE1 | 2 |
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 |
| NCT05918302 | PHASE3 | RECRUITING | Efficacy and Safety of Radiotherapy Compared to Everolimus in Somatostatin Receptor Positive Neuroendocrine Tumors of the Lung and Thymus. |
| NCT02834013 | PHASE2 | ACTIVE_NOT_RECRUITING | Nivolumab and Ipilimumab in Treating Patients With Rare Tumors |
| NCT03110978 | PHASE2 | ACTIVE_NOT_RECRUITING | Stereotactic Body Radiation Therapy With or Without Nivolumab in Treating Patients With Stage I-IIA or Recurrent Non-small Cell Lung Cancer |
| NCT03600233 | PHASE2 | ACTIVE_NOT_RECRUITING | Study of CVM-1118 for Patients With Advanced Neuroendocrine Tumors |
| NCT04665739 | PHASE2 | RECRUITING | Testing Lutetium Lu 177 Dotatate in Patients With Somatostatin Receptor Positive Advanced Bronchial Neuroendocrine Tumors |
| NCT04893785 | PHASE2 | RECRUITING | A Trial Evaluating the Activity and Safety of Combination Between Cabozantinib and Temozolomide in Lung and GEP-NENS Progressive After Everolimus, Sunitinib or PRRT (CABOTEM) |
| NCT06202066 | PHASE2 | RECRUITING | Temozolomide and Survivin Long Peptide Vaccine (SurVaxM) for the Treatment of Patients With Progressing Metastatic Neuroendocrine Carcinomas |
| NCT03095274 | PHASE2 | COMPLETED | Durvalumab (MEDI4736) Plus Tremelimumab for Advanced Neuroendocrine Neoplasms of Gastroenteropancreatic or Lung Origin |
| NCT04276597 | PHASE2 | WITHDRAWN | Phase-II Study of Lu177DOTATOC in Adults With STTR(+)Pulmonary, Pheochromocytoma, Paraganglioma, Unknown Primary, Thymus NETs (PUTNET), or Any Other Non-.GEP-NET. |
| NCT02589522 | PHASE1 | ACTIVE_NOT_RECRUITING | Testing the Safety of M6620 (VX-970) When Given With Standard Whole Brain Radiation Therapy for the Treatment of Brain Metastases From Non-small Cell Lung Cancer, Small Cell Lung Cancer, or Neuroendocrine Tumors |
| NCT01324492 | PHASE1 | COMPLETED | Safety and Efficacy of RAD001 in Chinese Patients With Advanced Pulmonary Neuroendocrine Tumor |
| NCT05064150 | Not specified | ACTIVE_NOT_RECRUITING | Neuroendocrine Tumors - Patient Reported Outcomes |
| NCT06282016 | Not specified | RECRUITING | Risk Factors for Neuroendocrine Neoplasms |
| NCT06402695 | Not specified | RECRUITING | Observational Study on GEP-and Pulm-NET Treated at FPG |
| NCT06523582 | Not specified | RECRUITING | Genetic Bases of Neuroendocrine Neoplasms in Mexican Patients |
| NCT02948946 | Not specified | COMPLETED | Clinical Utility Assay as a Biomarker for Gastroenteropancreatic and Lung Neuroendocrine Tumors |
| NCT03033186 | Not specified | UNKNOWN | Everolimus TDM to Predict Long Term Toxicity |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| CABOZANTINIB | 4 | 5 |
| FLUDEOXYGLUCOSE F 18 | 4 | 1 |
| LUTETIUM OXODOTREOTIDE LU-177 | 4 | 1 |
| TREMELIMUMAB | 4 | 1 |
| BERZOSERTIB | 2 | 1 |
| FOSLINANIB | 2 | 1 |
| LUTETIUM LU177 EDOTREOTIDE | 2 | 1 |
| SVN53-67/M57-KLH PEPTIDE VACCINE | 2 | 1 |