◎ Sugi Atlas

Atlas / Disease / Neuroendocrine carcinoma

Neuroendocrine carcinoma

disease
On this page

Also known as NECneuroendocrine cancer

Summary

Neuroendocrine carcinoma (MONDO:0002120) is a cancer (an umbrella term covering 12 Mondo subtypes) and 114 clinical trials. Top therapeutic interventions include irinotecan, dostarlimab, and edotreotide gallium ga-68. A subtype of carcinoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

  • Classification: Cancer
  • Umbrella term: 12 Mondo subtypes
  • Clinical trials: 114

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

FieldValue
Canonical nameneuroendocrine carcinoma
Mondo IDMONDO:0002120
MeSHD018278
DOIDDOID:1800
NCITC3773
SNOMED CT253000007
UMLSC0206695
MedGen104919
GARD0023067
Is cancer (heuristic)yes

Also known as: NEC · neuroendocrine cancer · neuroendocrine carcinoma

Disease family

This is a subtype of carcinoma. 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 › cancer › carcinoma › neuroendocrine carcinoma

Related subtypes (48): retroperitoneum carcinoma, head and neck carcinoma, peritoneal carcinoma, laryngeal carcinoma, bone carcinoma, carcinoma ex pleomorphic adenoma, scrotal carcinoma, skin carcinoma, malignant myoepithelioma, trachea carcinoma, epithelial-myoepithelial carcinoma, lipid-rich carcinoma, comedocarcinoma, in situ carcinoma, adenocarcinoma, urinary bladder carcinoma, breast carcinoma, squamous cell carcinoma, lung carcinoma, prostate carcinoma, renal carcinoma, uterine carcinoma, vulvar carcinoma, large cell carcinoma, undifferentiated carcinoma, basaloid carcinoma, cribriform carcinoma, digestive system carcinoma, fallopian tube carcinoma, penile carcinoma, sarcomatoid carcinoma, thymic carcinoma, transitional cell carcinoma, ureter carcinoma, papillary carcinoma, Krebs 2 carcinoma, thyroid gland carcinoma, vaginal carcinoma, choroid plexus carcinoma, malignant epithelial tumor of ovary, mucin-producing carcinoma, basal cell carcinoma, carcinoma of urethra, combined carcinoid and adenocarcinoma, secondary carcinoma, invasive carcinoma, glycogen-rich carcinoma, lymph node carcinoma

Subtypes (12): small cell carcinoma, anal canal neuroendocrine neoplasm, large cell neuroendocrine carcinoma, pancreatic endocrine carcinoma, combined lung carcinoma, malignant adrenal gland pheochromocytoma, liver neuroendocrine carcinoma, medullary thyroid gland carcinoma, goblet cell carcinoma, cutaneous neuroendocrine carcinoma, thymic neuroendocrine carcinoma, vulvar neuroendocrine carcinoma

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

0 approved, 4 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.

DrugDevelopment status
FluorouracilPhase 3 (in late-stage trials)
IrinotecanPhase 3 (in late-stage trials)
SurufatinibPhase 3 (in late-stage trials)
ToripalimabPhase 3 (in late-stage trials)

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Atezolizumab, Avelumab, Bevacizumab, Cabozantinib, Camrelizumab, Capecitabine, Carboplatin, Carfilzomib, Cisplatin, Durvalumab, Etoposide, Everolimus, Gimeracil, Ipilimumab, Lenvatinib, Melphalan, Nivolumab, Octreotide, Oteracil, Oxaliplatin, Paclitaxel, Pembrolizumab, Pertuzumab, Rivoceranib, Sintilimab, Spartalizumab, Tegafur, Temozolomide, Tucidinostat.

Clinical trials & evidence

Clinical trials

Clinical trials: 114.

Phase distribution (across all retrieved trials)

PhaseTrials
PHASE254
Not specified26
PHASE1/PHASE215
PHASE113
PHASE33
EARLY_PHASE12
PHASE2/PHASE31

Top trials by phase / activity

NCTPhaseStatusTitle
NCT06937905PHASE3RECRUITINGTarlatamab vs Standard of Care Chemotherapy in Patients With Pre-treated Advanced, Pulmonary or Gastroenteropancreatic Poorly Differentiated Neuroendocrine Carcinomas (NECs)
NCT07595913PHASE3NOT_YET_RECRUITINGA Study of ZG006 for Injection Versus Investigator-Selected Chemotherapy in Participants With Advanced Neuroendocrine Carcinoma
NCT01263041PHASE2/PHASE3COMPLETEDEffect of L-arginine and Glutamine on Preterm
NCT05015621PHASE3UNKNOWNA Phase 3 Study to Evaluate Surufatinib Plus Toripalimab in the Treatment of Advanced Neuroendocrine Carcinoma
NCT03074513PHASE2ACTIVE_NOT_RECRUITINGAtezolizumab and Bevacizumab in Treating Patients With Rare Solid Tumors
NCT03110978PHASE2ACTIVE_NOT_RECRUITINGStereotactic Body Radiation Therapy With or Without Nivolumab in Treating Patients With Stage I-IIA or Recurrent Non-small Cell Lung Cancer
NCT03600233PHASE2ACTIVE_NOT_RECRUITINGStudy of CVM-1118 for Patients With Advanced Neuroendocrine Tumors
NCT03647163PHASE1/PHASE2ACTIVE_NOT_RECRUITINGPh I/II Trial of Systemic VSV-IFNβ-NIS in Combination With Checkpoint Inhibitor Therapy in Patients With Select Solid Tumors
NCT03728361PHASE2ACTIVE_NOT_RECRUITINGNivolumab and Temozolomide in Treating Patients With Recurrent or Refractory Small-Cell Lung Cancer or Advanced Neuroendocrine Cancer
NCT04079712PHASE2ACTIVE_NOT_RECRUITINGTesting the Combination of XL184 (Cabozantinib), Nivolumab, and Ipilimumab for Poorly Differentiated Neuroendocrine Tumors
NCT04268121PHASE2RECRUITINGEfficacy of Neoadjuvant Chemotherapy in Terms of DFS in Patients With Localized Digestive Neuroendocrine Carcinomas
NCT04325425PHASE2RECRUITINGChemotherapy For Metastatic Grade 3 Poorly Differentiated NEuroendocrine Carcinoma Of GastroEnteroPancreatic And Unknown Primary
NCT04471727PHASE1/PHASE2ACTIVE_NOT_RECRUITINGA Study in Participants With Advanced Cancers Associated With Expression of DLL3 (MK-6070-001/HPN328-4001)
NCT04524208PHASE2ACTIVE_NOT_RECRUITINGA Trial of Cabozantinib in Patients With Advanced, Low Proliferative NEN G3
NCT04701307PHASE2ACTIVE_NOT_RECRUITINGNiraparib and Dostarlimab for the Treatment of Small Cell Lung Cancer and Other High-Grade Neuroendocrine Carcinomas
NCT05396118PHASE2ENROLLING_BY_INVITATIONSimultaneous Hyperpolarized [1-13C]Pyruvate and 18F-FDG PET/MRS in Cancer Patients
NCT05969860PHASE2RECRUITINGAt-Home Cancer Directed Therapy Versus in Clinic for the Treatment of Patients With Advanced Cancer
NCT05978284PHASE1/PHASE2RECRUITINGStudy of ZG006 in Participants With Small Cell Lung Cancer or Neuroendocrine Carcinoma
NCT06157827PHASE1/PHASE2RECRUITINGA Clinical Trial of LBL-024 Combined With Etoposide and Platinum in Patients With Advanced Neuroendocrine Carcinoma
NCT06283719PHASE1/PHASE2RECRUITINGDose Escalation Study of ZG006 in Participants With Advanced Small Cell Lung Cancer or Neuroendocrine Carcinoma, Followed by Dose Expansion Study in Participants With Advanced Small Cell Lung Cancer
NCT06333314PHASE2RECRUITINGDostarlimab for Locally Advanced or Metastatic Cancer (Non-colorectal/Non-endometrial) With Tumor dMMR/MSI
NCT06372626PHASE1/PHASE2RECRUITINGStudy of ZG005 in Combination With Etoposide and Cisplatin in Participants With Advanced Neuroendocrine Carcinoma.
NCT06440057PHASE1/PHASE2RECRUITINGDose Escalation Study of ZG006 in Participants With Advanced Small Cell Lung Cancer or Neuroendocrine Carcinoma, Followed by Dose Expansion Study in Participants With Neuroendocrine Carcinoma.
NCT06683846PHASE2RECRUITINGIvonescimab in the Treatment of Multiple Advanced Tumors
NCT06943521PHASE1/PHASE2RECRUITINGA Study of MT-4561 in Patients With Various Advanced Solid Tumors
NCT07038096PHASE1/PHASE2RECRUITINGZG006 and ZG005 in Participants With Small Cell Lung Cancer or Neuroendocrine Carcinoma
NCT07174583PHASE1/PHASE2RECRUITINGA Study of IDE849 in Patients With DLL3 Expressing Tumors Including Small Cell Lung Cancer
NCT07520357PHASE2NOT_YET_RECRUITINGA Phase II Clinical Study of SHR-A1904 Monotherapy in Second-Line or Later Treatment of Advanced Neuroendocrine Carcinoma
NCT00003514PHASE2WITHDRAWNAntineoplaston Therapy in Treating Patients With Neuroendocrine Tumor That Is Metastatic or Unlikely to Respond to Surgery or Radiation Therapy
NCT00004922PHASE2COMPLETEDIrinotecan in Treating Patients With Advanced Neuroendocrine Tumors
NCT00019786PHASE2COMPLETEDIsolated Hepatic Perfusion With Melphalan in Treating Patients With Primary Unresectable Liver Cancer or Liver Metastases
NCT00113360PHASE2COMPLETEDRAD001 Plus Octreotide Depot in Metastatic or Unresectable Low Grade Neuroendocrine Carcinoma
NCT00178698PHASE2UNKNOWNHyperthermia/Thermal Therapy With Chemotherapy to Treat Inoperable or Metastatic Tumors
NCT00193531PHASE2COMPLETEDPaclitaxel, Carboplatin, and Oral Etoposide Followed by Weekly Paclitaxel in High Grade Neuroendocrine Carcinoma
NCT00363051PHASE2COMPLETEDSafety/Efficacy of Everolimus in Adults With Advanced Pancreatic Neuroendocrine Cancer Not Responsive to Chemotherapy
NCT00388063PHASE2COMPLETEDSafety and Efficacy of Atiprimod Treatment for Patients With Low to Intermediate Grade Neuroendocrine Carcinoma
NCT00607113PHASE2COMPLETEDAvastin (Bevacizumab) and RAD001 (Everolimus) in Advanced Low or Intermediate Grade Neuroendocrine Carcinoma
NCT00663429PHASE2COMPLETEDExtension Study of the Safety and Efficacy of Atiprimod Treatment in Neuroendocrine Carcinoma
NCT01121939PHASE2COMPLETEDCombination of Bevacizumab, Pertuzumab, and Sandostatin for Adv. Neuroendocrine Cancers
NCT01203306PHASE2UNKNOWNBevacizumab Plus Somatostatin Analogue and Metronomic Capecitabine in Patients With Advanced Neuroendocrine Tumors

Drugs tested across these trials (top 30)

MoleculeMax phaseTrials referencing
IRINOTECAN43
DOSTARLIMAB42
EDOTREOTIDE GALLIUM GA-6842
FLUDEOXYGLUCOSE F 1842
FLUPHENAZINE DECANOATE42
OCTREOTIDE42
AVELUMAB41
CABOZANTINIB S-MALATE41
CARFILZOMIB41
COPPER OXODOTREOTIDE CU-6441
GLUTAMINE41
INDIUM IN 111 PENTETREOTIDE41
LENVATINIB41
NIRAPARIB41
PERTUZUMAB41
TARLATAMAB41
SURUFATINIB32
TUCIDINOSTAT32
VELIPARIB32
ANTINEOPLASTON A1031
ARGININE31
L-CITRULLINE31
SERPLULIMAB31
ATIPRIMOD24
IADADEMSTAT22
VUDALIMAB22
FOSLINANIB21
GOCATAMIG21
LUTETIUM LU177 EDOTREOTIDE21
ZENIDOLOL21

About this page

Generated deterministically by Sugi Atlas from primary biomedical databases via BioBTree. Every record on this page traces to a primary source.

Generated
Atlas version
v1.1.2
BioBTree
v2.0.2

Sources 21

This page pulls from 21 datasets indexed by BioBTree:

chembl_moleculecivic_evidenceclinical_trialsclinvardoidefogardgenccgwasgwas_studyhgncmedgenmeshmimmondomondochildmondoparentncitorphanetsctidumls