Lung carcinoid tumor

Summary

Lung carcinoid tumor (MONDO:0006041) is a cancer with 1 cohort gene (1 CIViC-evidence somatic driver; 1 ClinVar predisposition record) and 12 clinical trials. Top therapeutic interventions include temsirolimus, octreotide acetate, and pazopanib hydrochloride.

At a glance

• Classification: Cancer
• Cohort genes: 1
• ClinVar variants: 1
• Clinical trials: 12

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	lung carcinoid tumor
Mondo ID	MONDO:0006041
EFO	EFO:1000037
NCIT	C4038
SNOMED CT	254627002
UMLS	C0280089
MedGen	79070
GARD	0024273
Anatomy (UBERON)	UBERON:0002048
Is cancer (heuristic)	yes

Also known as: carcinoid tumor (disease) of lung · carcinoid tumor of lung · carcinoid tumor of the lung · carcinoid tumour (disease) of lung · carcinoid tumour of lung · carcinoid tumour of the lung · lung carcinoid · lung carcinoid tumor · lung carcinoid tumor (disease) · lung carcinoid tumour (disease) · pulmonary carcinoid tumor · pulmonary carcinoid tumour

Data availability: 1 ClinVar variant · 6 cell lines.

Disease family

Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › endocrine gland neoplasm › neuroendocrine neoplasm › carcinoid tumor › lung carcinoid tumor

Related subtypes (6): atypical carcinoid tumor, gastric neuroendocrine tumor G1, somatostatinoma, intestinal neuroendocrine tumor G1, pancreatic neuroendocrine tumor G1, childhood carcinoid 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

ClinVar germline variants

1 retrieved; paginated sample, class counts are floors:

1 pathogenic

ClinVar	Variant (HGVS)	Gene	Classification	Review
279852	NM_001370259.2(MEN1):c.1546dup (p.Arg516fs)	MEN1	Pathogenic	criteria provided, multiple submitters, no conflicts

Genes & proteins

Mendelian disease overlap and somatic drivers

GenCC: 0 · Orphanet: 7 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0

Somatic driver evidence (intOGen + CIViC, cohort fanout)

Gene	intOGen role	Cancer types	CIViC
MEN1	LoF	ACC,BLCA,BRCA,HCC,LUNG,PANCREAS,PANET,WDTC	CIViC #3485

Orphanet rare-disease linkage (cohort genes)

Gene	Orphanet ID	Rare disease
MEN1	Orphanet:2965	Prolactinoma
MEN1	Orphanet:314786	Silent pituitary adenoma
MEN1	Orphanet:314790	Null pituitary adenoma
MEN1	Orphanet:652	Multiple endocrine neoplasia type 1
MEN1	Orphanet:97279	Insulinoma
MEN1	Orphanet:99725	Pituitary gigantism
MEN1	Orphanet:99879	Familial isolated hyperparathyroidism

Cohort genes → proteins

1 cohort genes, 1 distinct canonical proteins.

Evidence partition

Subset	Genes
multi_evidence	1

Cohort genes (full)

Symbol	HGNC	Ensembl	UniProt	Name	Evidence
MEN1	HGNC:7010	ENSG00000133895	O00255	Menin	clinvar

Cohort function summary

Lead sentence per gene, UniProt-curated.

Symbol	Protein name	Function (lead sentence)
MEN1	Menin	Essential component of a MLL/SET1 histone methyltransferase (HMT) complex, a complex that specifically methylates ‘Lys-4’ of histone H3 (H3K4).

Protein-family classification

Druggable: 0 · Difficult: 0 · Unknown: 1 · Druggable fraction: 0.0

Family distribution

Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.

Family	Genes	Fold	FDR
Other/Unknown	1	1.8×	0.558

Per-gene assignment

Symbol	Family	Druggable?	EC	InterPro (top 3)
MEN1	Other/Unknown	no		Menin

Expression context

Cohort genes with no expression data: 0.

1 cohort gene are a single-cell marker in ≥1 SCXA experiment.

Breadth distribution (Bgee present_calls)

Bucket	Genes
narrow (1-5 tissues)	0
moderate (6-20)	0
broad (>20)	1
unknown	0

Top tissues across cohort

Tissue	Cohort genes
granulocyte	1
lower esophagus mucosa	1
right hemisphere of cerebellum	1

Per-gene tissue summary (top 30)

Symbol	Bgee breadth	FANTOM5 breadth	SCXA	Top tissues
MEN1	271	ubiquitous	marker	granulocyte, lower esophagus mucosa, right hemisphere of cerebellum

Protein interactions among cohort

Intra-cohort edges: 0.

Hub genes (top 10 by interactor count)

Symbol	Interactor count
MEN1	5,226

Structural data

PDB: 1 · AlphaFold-only: 0 · No structure: 0

Cohort genes with PDB structures (top 30)

Symbol	UniProt	PDB entries
MEN1	O00255	69

Function

Pathway analysis

Distinct Reactome pathways touched by cohort: 24. Enrichment computed across 1 evidence-associated genes (1 with Reactome annotation).

Pathways by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

Pathway	Cohort genes	Fold	FDR	Sample cohort genes
Transcriptional activity of SMAD2/SMAD3:SMAD4 heterotrimer	1	368.4×	0.018	MEN1
RHO GTPases activate IQGAPs	1	346.1×	0.018	MEN1
SMAD2/SMAD3:SMAD4 heterotrimer regulates transcription	1	308.6×	0.018	MEN1
Formation of WDR5-containing histone-modifying complexes	1	265.6×	0.018	MEN1
Deactivation of the beta-catenin transactivating complex	1	233.1×	0.018	MEN1
Signaling by TGF-beta Receptor Complex	1	200.3×	0.018	MEN1
Epigenetic regulation by WDR5-containing histone modifying complexes	1	154.3×	0.018	MEN1
Differentiation of naive CD4+ T cells to T helper 2 cells (Th2 cells)	1	146.4×	0.018	MEN1
Formation of the beta-catenin:TCF transactivating complex	1	120.2×	0.018	MEN1
TCF dependent signaling in response to WNT	1	117.7×	0.018	MEN1
Signaling by TGFB family members	1	115.3×	0.018	MEN1
Signaling by WNT	1	112.0×	0.018	MEN1
Post-translational protein phosphorylation	1	100.2×	0.018	MEN1
Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)	1	86.5×	0.020	MEN1
Epigenetic regulation of gene expression	1	71.4×	0.022	MEN1
RHO GTPase Effectors	1	68.0×	0.022	MEN1
Signaling by Rho GTPases	1	34.2×	0.040	MEN1
Signaling by Rho GTPases, Miro GTPases and RHOBTB3	1	33.5×	0.040	MEN1
RNA Polymerase II Transcription	1	22.5×	0.056	MEN1
Post-translational protein modification	1	19.2×	0.063	MEN1
Gene expression (Transcription)	1	17.8×	0.064	MEN1
Generic Transcription Pathway	1	15.1×	0.072	MEN1
Metabolism of proteins	1	12.4×	0.084	MEN1
Signal Transduction	1	10.2×	0.098	MEN1

GO biological processes by enrichment

Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.

GO term	Cohort genes	Fold	FDR	Sample cohort genes
negative regulation of cyclin-dependent protein serine/threonine kinase activity	1	2106.5×	0.005	MEN1
T-helper 2 cell differentiation	1	1872.4×	0.005	MEN1
osteoblast development	1	991.3×	0.005	MEN1
obsolete negative regulation of DNA-binding transcription factor activity	1	732.7×	0.005	MEN1
negative regulation of protein phosphorylation	1	581.1×	0.005	MEN1
response to gamma radiation	1	581.1×	0.005	MEN1
negative regulation of JNK cascade	1	561.7×	0.005	MEN1
positive regulation of transforming growth factor beta receptor signaling pathway	1	526.6×	0.005	MEN1
transcription initiation-coupled chromatin remodeling	1	383.0×	0.005	MEN1
response to UV	1	366.4×	0.005	MEN1
negative regulation of osteoblast differentiation	1	295.6×	0.006	MEN1
negative regulation of cell cycle	1	290.6×	0.006	MEN1
MAPK cascade	1	153.2×	0.010	MEN1
DNA repair	1	63.8×	0.022	MEN1
DNA damage response	1	53.5×	0.025	MEN1
negative regulation of cell population proliferation	1	42.1×	0.030	MEN1
negative regulation of DNA-templated transcription	1	31.6×	0.037	MEN1
negative regulation of transcription by RNA polymerase II	1	17.7×	0.063	MEN1
positive regulation of transcription by RNA polymerase II	1	14.9×	0.071	MEN1
regulation of transcription by RNA polymerase II	1	11.7×	0.086	MEN1

Therapeutics

Drug target analysis

Approved (phase 4): 1 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 0

Druggability breadth: 1 of 1 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).

Genes with an approved drug

The molecule shown is one approved compound that hits the gene — not necessarily a drug of choice or one indicated for this disease.

Symbol	Example approved molecule
MEN1	LOPERAMIDE

Top cohort targets by molecule count

Symbol	Molecules	Max phase
MEN1	475	4

Drugs targeting cohort genes (top 30)

Molecule	Max phase	Targets in cohort
LOPERAMIDE	4	MEN1
CANDESARTAN CILEXETIL	4	MEN1
EVANS BLUE FREE ACID	4	MEN1
DIENESTROL	4	MEN1
BEXAROTENE	4	MEN1
IFOSFAMIDE	4	MEN1
PROGESTERONE	4	MEN1
CLOTRIMAZOLE	4	MEN1
AMINOCAPROIC ACID	4	MEN1
LATANOPROST	4	MEN1
FLUORESCEIN	4	MEN1
OXCARBAZEPINE	4	MEN1
SALMETEROL XINAFOATE	4	MEN1
AMIODARONE HYDROCHLORIDE	4	MEN1
TRICLABENDAZOLE	4	MEN1
TRYPAN BLUE FREE ACID	4	MEN1
MIGALASTAT	4	MEN1
DROPERIDOL	4	MEN1
ARIPIPRAZOLE	4	MEN1
AMOXAPINE	4	MEN1
RALOXIFENE HYDROCHLORIDE	4	MEN1
IDARUBICIN	4	MEN1
ACETAMINOPHEN	4	MEN1
OXYBUTYNIN CHLORIDE	4	MEN1
DECAMETHONIUM BROMIDE	4	MEN1
DESLORATADINE	4	MEN1
DITHIAZANINE	4	MEN1
TRIMETREXATE	4	MEN1
NICARDIPINE HYDROCHLORIDE	4	MEN1
PROTRIPTYLINE HYDROCHLORIDE	4	MEN1

Bioactivity and enzyme data

Enzyme cohort genes (≥1 EC): 0.

Cohort genes with ChEMBL bioactivity (full, sorted by assay count)

Symbol	Assays	Type breakdown
MEN1	93	Binding:86, Functional:7

Pharmacogenomics

Cohort genes with a PharmGKB record: 1; with CPIC/DPWG dosing guidelines: 0.

No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).

Drug repurposing candidates

30 approved/phased drugs hit cohort targets but don’t yet appear in disease-level clinical trials. Target-inhibition rationale is strongest for cancer driver genes; a bioactivity hit is a screening signal, not a treatment claim.

Compound	Max phase	Cohort target (bioactivity)
LOPERAMIDE	4	MEN1
CANDESARTAN CILEXETIL	4	MEN1
EVANS BLUE FREE ACID	4	MEN1
DIENESTROL	4	MEN1
BEXAROTENE	4	MEN1
IFOSFAMIDE	4	MEN1
PROGESTERONE	4	MEN1
CLOTRIMAZOLE	4	MEN1
AMINOCAPROIC ACID	4	MEN1
LATANOPROST	4	MEN1
FLUORESCEIN	4	MEN1
OXCARBAZEPINE	4	MEN1
SALMETEROL XINAFOATE	4	MEN1
AMIODARONE HYDROCHLORIDE	4	MEN1
TRICLABENDAZOLE	4	MEN1
TRYPAN BLUE FREE ACID	4	MEN1
MIGALASTAT	4	MEN1
DROPERIDOL	4	MEN1
ARIPIPRAZOLE	4	MEN1
AMOXAPINE	4	MEN1
RALOXIFENE HYDROCHLORIDE	4	MEN1
IDARUBICIN	4	MEN1
ACETAMINOPHEN	4	MEN1
OXYBUTYNIN CHLORIDE	4	MEN1
DECAMETHONIUM BROMIDE	4	MEN1
DESLORATADINE	4	MEN1
DITHIAZANINE	4	MEN1
TRIMETREXATE	4	MEN1
NICARDIPINE HYDROCHLORIDE	4	MEN1
PROTRIPTYLINE HYDROCHLORIDE	4	MEN1

Druggability pyramid

Cohort genes binned by druggability tier (high → low):

Tier	Definition	Genes	Symbols
A	Approved (phase 4 drug)	1	MEN1
B	Phased (≥1) drug, not yet approved	0
C	Druggable family + PDB, no drug	0
D	Druggable family + AlphaFold only, no drug	0
E	Difficult family or no structure, no drug	0

Undrugged target profiles

0 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).

Clinical trials & evidence

Clinical trials

Clinical trials: 12.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	6
PHASE1	3
Not specified	3

Top trials by phase / activity

NCT	Phase	Status	Title
NCT00084461	PHASE2	TERMINATED	Romidepsin in Treating Patients With Locally Advanced or Metastatic Neuroendocrine Tumors
NCT00093782	PHASE2	COMPLETED	Temsirolimus in Treating Patients With Metastatic Neuroendocrine Carcinoma
NCT00454363	PHASE2	COMPLETED	Pazopanib Hydrochloride in Treating Patients With Advanced Neuroendocrine Cancer
NCT01010126	PHASE2	COMPLETED	Temsirolimus and Bevacizumab in Treating Patients With Advanced Endometrial, Ovarian, Liver, Carcinoid, or Islet Cell Cancer
NCT02259725	PHASE2	COMPLETED	Regorafenib in Treating Patients With Advanced or Metastatic Neuroendocrine Tumors
NCT04915144	PHASE2	WITHDRAWN	177Lu-DOTATOC for the Treatment of Patients With Somatostatin Receptor Positive NETs
NCT00004074	PHASE1	COMPLETED	Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu
NCT01204476	PHASE1	COMPLETED	Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma
NCT01548482	PHASE1	COMPLETED	Trebananib And Temsirolimus in Treating Patients With Solid Tumors That Are Metastatic or Cannot Be Removed by Surgery
NCT03707925	Not specified	TERMINATED	Bronchoscopic Laser Ablation of Peripheral Lung Tumors
NCT03923777	Not specified	UNKNOWN	Active Surveillance in Early Lung Cancer
NCT04085081	Not specified	WITHDRAWN	Physical Activity Intervention Before and After Surgery in Older Adults With Lung Cancer and Their Family Caregivers

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
TEMSIROLIMUS	4	3
OCTREOTIDE ACETATE	4	1
PAZOPANIB HYDROCHLORIDE	4	1
REGORAFENIB	4	1
ROMIDEPSIN	4	1
TREBANANIB	3	1
CIXUTUMUMAB	2	1
EDODEKIN ALFA	2	1
LUTETIUM LU177 EDOTREOTIDE	2	1
CHEMBL4066465	0	1