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A malignant neuroendocrine neoplasm composed of cells containing secretory granules that stain positive for NSE and chromogranin. The neoplastic cells are often round and form clusters or trabecular sheets. Representative examples are small cell carcinoma, large cell neuroendocrine carcinoma, and Merkel cell carcinoma.
Biomarker and diagnostic research for neuroendocrine carcinoma has been reported in the published literature.
No approved treatments are currently available for neuroendocrine carcinoma. An additional 5 compounds hold orphan drug designation.
While no drugs are FDA-approved specifically for neuroendocrine carcinoma, some of the following designated compounds may be used off-label in clinical practice. Treatment decisions should be made in consultation with a specialist familiar with this condition.
The following drugs have received orphan drug designation from the FDA for neuroendocrine carcinoma. Orphan designation reflects regulatory interest and does not indicate approval for treatment.
Brand Name | Generic Name | Sponsor |
|---|
113 clinical trials registered, 31 recruiting. Interventions under study include drug therapy, biologic therapy, other interventions, and procedural interventions. Pipeline includes 1 PHASE4, 4 PHASE3, 48 PHASE2. Research is sponsored by a mix of industry and academic institutions.
NCT ID | Title | Phase | Sponsor | Status |
|---|---|---|---|---|
[NCT06176989](https://clinicaltrials.gov/study/NCT06176989) |
Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 6:00 AM UTC
Patient Advocacy Groups (PAGs) provide support, resources, and community for patients and caregivers.
Designated
Exclusivity End |
|---|
Designation Status |
|---|
a trispecific antibody binding to two different epitopes on tumor antigen DLL3 and tumor antigen CD3 | a trispecific antibody binding to two different epitopes on tumor antigen DLL3 and tumor antigen CD3 | Suzhou Zelgen Biopharmaceuticals Co.,Ltd | 2025 | — | Designated |
a humanized bispecific monoclonal antibody against PD-L1 and 4-1BB | a humanized bispecific monoclonal antibody against PD-L1 and 4-1BB | Nanjing Leads Biolabs Co., Ltd. | 2024 | — | Designated |
225Ac, an alpha emitting radionuclide, coordinated to the humanized monoclonal antibody (mAb) targeting the DLL3 receptor, along with a linker chelator conjugated to the mAb | 225Ac, an alpha emitting radionuclide, coordinated to the humanized monoclonal antibody (mAb) targeting the DLL3 receptor, along with a linker chelator conjugated to the mAb | Abdera Therapeutics | 2024 | — | Designated |
humanized IgG-like T cell engager (TcE) comprised of 2 polypeptide chains specific for human Delta-like 3 (DLL3) and human CD3 | humanized IgG-like T cell engager (TcE) comprised of 2 polypeptide chains specific for human Delta-like 3 (DLL3) and human CD3 | Boehringer Ingelheim Pharmaceuticals, Inc. | 2024 | — | Designated |
bispecific antibody targeting DLL3 and CD47 | bispecific antibody targeting DLL3 and CD47 | Phanes Therapeutics, Inc. | 2024 | — | Designated |
Gene therapy approaches for neuroendocrine carcinoma have been reported in the published literature.
113 trials found
Enasidenib in IDH2-Mutated Malignant Sinonasal and Skull Base Tumors |
PHASE2 |
National Cancer Institute (NCI) |
RECRUITING |
[NCT07174583](https://clinicaltrials.gov/study/NCT07174583) | A Study of IDE849 in Patients With DLL3 Expressing Tumors Including Small Cell Lung Cancer | PHASE1 | IDEAYA Biosciences | RECRUITING |
[NCT06070740](https://clinicaltrials.gov/study/NCT06070740) | First- Line Treatment With Durvalumab Plus XELOX Chemotherapy in Advanced Gastrointestinal Neuroendocrine Carcinoma | PHASE2 | Peking Union Medical College Hospital | RECRUITING |
[NCT06937905](https://clinicaltrials.gov/study/NCT06937905) | Tarlatamab vs Standard of Care Chemotherapy in Patients With Pre-treated Advanced, Pulmonary or Gastroenteropancreatic Poorly Differentiated Neuroendocrine Carcinomas (NECs) | PHASE3 | Intergroupe Francophone de Cancerologie Thoracique | RECRUITING |
[NCT05969860](https://clinicaltrials.gov/study/NCT05969860) | At-Home Cancer Directed Therapy Versus in Clinic for the Treatment of Patients With Advanced Cancer | PHASE2 | Mayo Clinic | RECRUITING |
384 publications have been identified in PubMed for neuroendocrine carcinoma. Research spans Case Report / Case Series (32%), Review / Meta-Analysis (22%), and Basic Science / Preclinical (13%).
Research Type | Count | % of Total |
|---|---|---|
Patient case studies | 123 | 32% |
Research summaries | 86 | 22% |
Laboratory research | 50 | 13% |
Disease patterns and progression | 39 | 10% |
Clinical study results | 36 | 9% |
Testing and diagnosis research | 32 | 8% |
Other research | 9 | 2% |
New treatment approaches | 9 | 2% |
Uccella S (2026). [PMID: 41273419](https://pubmed.ncbi.nlm.nih.gov/41273419/). *Virchows Archiv : an international journal of pathology*. [Epidemiology / Natural History]
Wong S (2026). [PMID: 41649408](https://pubmed.ncbi.nlm.nih.gov/41649408/). *Am J Dermatopathol*. [Diagnostic / Biomarker]
Tsukada A (2026). [PMID: 42208366](https://pubmed.ncbi.nlm.nih.gov/42208366/). *Lung Cancer*. [Review / Meta-Analysis]
Masumoto S (2026). [PMID: 41760240](https://pubmed.ncbi.nlm.nih.gov/41760240/). *Anticancer Res*. [Case Report / Case Series]
Ataka R (2026). [PMID: 41712531](https://pubmed.ncbi.nlm.nih.gov/41712531/). *Am J Case Rep*. [Case Report / Case Series]
Hu H (2026). [PMID: 41578491](https://pubmed.ncbi.nlm.nih.gov/41578491/). *Medicine (Baltimore)*. [Review / Meta-Analysis]
Chen X (2026). [PMID: 41790645](https://pubmed.ncbi.nlm.nih.gov/41790645/). *Medicine (Baltimore)*. [Epidemiology / Natural History]
Jia M (2026). [PMID: 41792048](https://pubmed.ncbi.nlm.nih.gov/41792048/). *Cancer Med*. [Diagnostic / Biomarker]
O'Rorke MA (2026). [PMID: 42043735](https://pubmed.ncbi.nlm.nih.gov/42043735/). *Endocrine*. [Epidemiology / Natural History]
Bagasariya R (2026). [PMID: 42264904](https://pubmed.ncbi.nlm.nih.gov/42264904/). *J Nucl Med Technol*. [Basic Science / Preclinical]
AI-curated news mentioning neuroendocrine carcinoma
Updated Sep 12, 2026
A multicenter study involving 1,380 neuroendocrine neoplasms reveals divergent c-MYC expression patterns between neuroendocrine tumors (NET) and neuroendocrine carcinomas (NEC). These findings could inform future research and therapeutic strategies targeting c-MYC in these diseases.