Pancreatic endocrine carcinoma

Summary

Pancreatic endocrine carcinoma (MONDO:0005893) is a cancer and 35 clinical trials. Top therapeutic interventions include octreotide, fluphenazine decanoate, and pazopanib hydrochloride. A subtype of neuroendocrine carcinoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 35

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	pancreatic endocrine carcinoma
Mondo ID	MONDO:0005893
EFO	EFO:0007416
MeSH	D018273
Orphanet	506098
DOID	DOID:1798
ICD-10-CM	C25.4
NCIT	C3770
SNOMED CT	254612002
UMLS	C1328479
MedGen	233070
GARD	0022056
Anatomy (UBERON)	UBERON:0000016
Is cancer (heuristic)	yes

Also known as: carcinoma of endocrine pancreas · carcinoma, islet cell, malignant · endocrine pancreas carcinoma · high grade pancreatic neuroendocrine carcinoma · high-grade pancreatic neuroendocrine carcinoma · islet cell cancer · islet cell carcinoma · islet cell carcinoma (morphologic abnormality) · malignant islet cell tumor · malignant islet cell tumour · malignant neoplasm of islets of Langerhans · malignant pancreatic endocrine tumor · malignant pancreatic endocrine tumour · pancreatic endocrine cancer · pancreatic endocrine carcinoma · pancreatic NEC · pancreatic NEC G3 · Pancreatic Neuroendocrine cancer · pancreatic neuroendocrine carcinoma · poorly differentiated pancreatic endocrine carcinoma (+4 more)

Disease family

This is a subtype of neuroendocrine 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 › pancreatic endocrine carcinoma

Related subtypes (11): small cell carcinoma, anal canal neuroendocrine neoplasm, large cell neuroendocrine 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

Subtypes (2): pancreatic large cell neuroendocrine carcinoma, pancreatic small cell 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

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Bevacizumab, Capecitabine, Carboplatin, Cisplatin, Etoposide, Filgrastim, Fluorouracil, Irinotecan, Oxaliplatin, Pegfilgrastim, Sunitinib, Temozolomide, Temsirolimus.

Clinical trials & evidence

Clinical trials

Clinical trials: 35.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	25
Not specified	5
PHASE1	4
PHASE1/PHASE2	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT01824875	PHASE2	ACTIVE_NOT_RECRUITING	Temozolomide With or Without Capecitabine in Treating Patients With Advanced Pancreatic Neuroendocrine Tumors
NCT02595424	PHASE2	ACTIVE_NOT_RECRUITING	Cisplatin and Etoposide Versus Temozolomide and Capecitabine in Patients With Advanced Poorly Differentiated (G3) Non-Small Cell Gastrointestinal Neuroendocrine Carcinomas
NCT02834013	PHASE2	ACTIVE_NOT_RECRUITING	Nivolumab and Ipilimumab in Treating Patients With Rare Tumors
NCT05988918	PHASE2	ACTIVE_NOT_RECRUITING	Multicenter Trial of ESK981 in Patients With Select Solid Tumors
NCT00003514	PHASE2	WITHDRAWN	Antineoplaston Therapy in Treating Patients With Neuroendocrine Tumor That Is Metastatic or Unlikely to Respond to Surgery or Radiation Therapy
NCT00075439	PHASE2	COMPLETED	Gefitinib in Treating Patients With Progressive Metastatic Neuroendocrine Tumors
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
NCT00113360	PHASE2	COMPLETED	RAD001 Plus Octreotide Depot in Metastatic or Unresectable Low Grade Neuroendocrine Carcinoma
NCT00131911	PHASE2	COMPLETED	Sorafenib Tosylate in Treating Patients With Progressive Metastatic Neuroendocrine Tumors
NCT00227773	PHASE2	WITHDRAWN	Vatalanib and Octreotide in Treating Patients With Progressive Neuroendocrine Tumors
NCT00363051	PHASE2	COMPLETED	Safety/Efficacy of Everolimus in Adults With Advanced Pancreatic Neuroendocrine Cancer Not Responsive to Chemotherapy
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
NCT01465659	PHASE1/PHASE2	COMPLETED	Temozolomide and Pazopanib Hydrochloride in Treating Patients With Advanced Pancreatic Neuroendocrine Tumors That Cannot Be Removed By Surgery
NCT01525082	PHASE2	COMPLETED	Capecitabine, Temozolomide, and Bevacizumab for Metastatic or Unresectable Pancreatic Neuroendocrine Tumors
NCT02031536	PHASE2	TERMINATED	Everolimus in Patients With Pancreatic Neuroendocrine Tumors Metastatic to the Liver Previously Treated With Surgery
NCT02101918	PHASE2	COMPLETED	Ziv-Aflibercept in Treating and Computed Tomography Perfusion Imaging in Predicting Response in Patients With Pancreatic Neuroendocrine Tumors That Are Metastatic or Cannot Be Removed by Surgery
NCT02108782	PHASE2	WITHDRAWN	Dovitinib Lactate in Treating Patients With Pancreatic Neuroendocrine Tumors
NCT02230176	PHASE2	COMPLETED	Antitumor Efficacy of Peptide Receptor Radionuclide Therapy With 177Lutetium -Octreotate Randomized vs Sunitinib in Unresectable Progressive Well-differentiated Neuroendocrine Pancreatic Tumor: First Randomized Phase II
NCT02259725	PHASE2	COMPLETED	Regorafenib in Treating Patients With Advanced or Metastatic Neuroendocrine Tumors
NCT02273752	PHASE2	TERMINATED	Pharmacokinetically Guided Everolimus in Patients With Breast Cancer, Pancreatic Neuroendocrine Tumors, or Kidney Cancer
NCT02806648	PHASE2	COMPLETED	A Trial to Assess the Activity and Safety of Palbociclib in Patients With Well and Moderately Differentiated Metastatic Pancreatic Neuroendocrine Tumors (pNET)
NCT02893930	PHASE2	COMPLETED	Sapanisertib in Treating Patients With Metastatic or Refractory Pancreatic Neuroendocrine Tumor That Cannot Be Removed by Surgery
NCT03042780	PHASE2	TERMINATED	FOLFIRINOX in Metastatic High Grade Gastroenteropancreatic Neuroendocrine Carcinomas
NCT03736720	PHASE2	TERMINATED	Liposomal Irinotecan, Fluorouracil and Leucovorin in Treating Patients With Refractory Advanced High Grade Neuroendocrine Cancer of Gastrointestinal, Unknown, or Pancreatic Origin
NCT00004074	PHASE1	COMPLETED	Interleukin-12 and Trastuzumab in Treating Patients With Cancer That Has High Levels of HER2/Neu
NCT00655655	PHASE1	COMPLETED	Everolimus and Vatalanib in Treating Patients With Advanced Solid Tumors
NCT01155258	PHASE1	COMPLETED	Temsirolimus and Vinorelbine Ditartrate in Treating Patients With Unresectable or Metastatic Solid Tumors
NCT01204476	PHASE1	COMPLETED	Cixutumumab, Everolimus, and Octreotide Acetate in Treating Patients With Advanced Low to Intermediate Grade Neuroendocrine Carcinoma
NCT00830557	Not specified	RECRUITING	Collecting Medical Information and Tissue Samples From Patients With Pancreatic Cancer or Other Pancreatic Disorders
NCT01365169	Not specified	RECRUITING	Association Between Health Care Provider (HCP)-Assessed ECOG Performance Status (PS) and Overall Survival, and Objectively Measure of Physical Activity (PA) Levels in Advance-cancer Patients
NCT01603004	Not specified	COMPLETED	Potential Molecular Predictors of Response to Novel Therapies in Metastatic Pancreatic Neuroendocrine Tumors
NCT03884179	Not specified	UNKNOWN	Diagnosis of PCL With EUS-FNA and Cross-sectional Imaging - A Report of Accuracy
NCT04483349	Not specified	COMPLETED	Assessment of What Patients and Healthcare Providers Value

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
OCTREOTIDE	4	4
FLUPHENAZINE DECANOATE	4	2
PAZOPANIB HYDROCHLORIDE	4	2
TEMSIROLIMUS	4	2
GEFITINIB	4	1
PALBOCICLIB	4	1
REGORAFENIB	4	1
ROMIDEPSIN	4	1
SORAFENIB	4	1
VATALANIB	3	4
ANTINEOPLASTON A10	3	1
CIXUTUMUMAB	2	1
DOVITINIB LACTATE	2	1
EDODEKIN ALFA	2	1
SAPANISERTIB	2	1
CHEMBL3350037	0	2
CHEMBL4066465	0	1
CHEMBL365847	0	1
CHEMBL3962632	0	1
CHEMBL3991933	0	1