General Information of Disease (ID: DISVGJET)

Disease Name Neuroendocrine cancer
Synonyms neuroendocrine carcinoma; neuroendocrine cancer; NEC
Disease Class 2B72: Stomach cancer
Definition
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.
Disease Hierarchy
DISH9F1N: Carcinoma
DISNPLOO: Neuroendocrine neoplasm
DISKTBM1: Malignant endocrine neoplasm
DISVGJET: Neuroendocrine cancer
ICD Code
ICD-11
ICD-11: 2B72.1
ICD-9
ICD-9: 209
Expand ICD-11
'2D4Y
Expand ICD-9
209
Disease Identifiers
MONDO ID
MONDO_0002120
MESH ID
D018278
UMLS CUI
C0206695
MedGen ID
104919
SNOMED CT ID
253000007

Drug-Interaction Atlas (DIA) of This Disease

Drug-Interaction Atlas (DIA)
This Disease is Treated as An Indication in 5 Approved Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
Ga 68 dotatate DMFIIDD Approved NA [1]
Iobenguane I-123 DM80OEZ Approved Small molecular drug [2]
Lutetium Lu 177 dotatate DMN1XVC Approved NA [3]
Octreotide DMHIDCJ Approved Small molecular drug [4]
Retifanlimab DMRKTCP Approved Antibody [5]
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This Disease is Treated as An Indication in 23 Clinical Trial Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
177-Lu-edotreotide DMFSHI6 Phase 3 Somatostatin analogue [6]
177-Lu-oxodotreotide DMMZS2T Phase 3 Small molecular drug [7]
177Lu-DOTA-octreotate DMP5L8S Phase 3 NA [8]
PDR001 DM53J6B Phase 3 Antibody [9]
PV-10 DM95UYC Phase 3 Small molecular drug [10]
Rova-T DMNGCAD Phase 3 NA [10]
RRx-001 DMQM2FH Phase 3 Small molecular drug [10]
Selinexor DMBD4K3 Phase 3 Small molecular drug [10]
Sulfatinib DMPOTM4 Phase 3 NA [11]
166Ho microspheres DME6VDB Phase 2 Radiopharmaceutical therapy agent [12]
CA4P DM9R0ZL Phase 2 NA [8]
CVM-1118 DMP86RS Phase 2 Small molecular drug [13]
Metaiodobenzylguanidine I-131 DMNST69 Phase 2 Small molecular drug [8]
ONC201 DMM5SCF Phase 2 NA [10]
SomaKit-TOC; gallium (68Ga) edotreotide DMDHON7 Phase 2 NA [8]
177Lu-labelled DOTA-JR11 DMVZ9XL Phase 1/2 Radiopharmaceutical therapy agent [14]
PEN-221 DMMAUOK Phase 1/2 NA [10]
SNX-5422 DMIU0VP Phase 1/2 Small molecular drug [10]
212Pb-labelled DOTAMTATE DMN9MPX Phase 1 Radiopharmaceutical therapy agent [15]
AlphaMedix DMBVM1Q Phase 1 Peptide [15]
AMG 757 DMLYP2N Phase 1 Antibody [16]
DP-1038 DMKLCLO Phase 1 NA [10]
XmAb18087 DMB3C0Q Phase 1 NA [10]
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⏷ Show the Full List of 23 Drug(s)
This Disease is Treated as An Indication in 2 Investigative Drug(s)
Drug Name Drug ID Highest Status Drug Type REF
98mTc-CIM-ANT DMJ1VAE Investigative NA [17]
99mTc-MIP-1407 DMYPD5L Investigative NA [18]
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Molecular Interaction Atlas (MIA) of This Disease

Molecular Interaction Atlas (MIA)
This Disease Is Related to 19 DTT Molecule(s)
Gene Name DTT ID Evidence Level Mode of Inheritance REF
ARF1 TT70KXY Limited Biomarker [19]
PAX5 TTA4REJ Limited Biomarker [20]
DPEP1 TTYUENF moderate Biomarker [21]
GLRX TTRJCNG moderate Biomarker [22]
GM2A TTGOFW6 moderate Genetic Variation [23]
GRIA2 TTWM461 moderate Altered Expression [24]
LY96 TT8S9AV moderate Genetic Variation [23]
PCSK2 TT46F0P moderate Biomarker [25]
RHBDF2 TTH1ZOP moderate Biomarker [26]
SQLE TTE14XG moderate Biomarker [27]
CD79B TTBN5I7 Strong Genetic Variation [28]
CDKN1B TTLGFVW Strong Altered Expression [29]
DLL3 TT1C9K6 Strong Biomarker [30]
PGRMC1 TTY3LAZ Strong Biomarker [31]
SST TTWF7UG Strong Biomarker [32]
CD274 TT8ZLTI Definitive Biomarker [33]
ELK3 TT5OJMV Definitive Altered Expression [34]
EPHB1 TT8MDAC Definitive Altered Expression [34]
SLC6A2 TTAWNKZ Definitive Altered Expression [34]
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⏷ Show the Full List of 19 DTT(s)
This Disease Is Related to 23 DOT Molecule(s)
Gene Name DOT ID Evidence Level Mode of Inheritance REF
CHGA OTXYX5JH Limited Biomarker [35]
TTF1 OT4K90WD Limited Biomarker [36]
DPT OTINRFC7 moderate Biomarker [37]
DSC2 OTODVH8K moderate Genetic Variation [38]
DSC3 OTYG47F8 moderate Genetic Variation [38]
INSM1 OTG8RV8E moderate Biomarker [39]
ISL1 OTVNVKAX moderate Biomarker [40]
NAPSA OT6F8IAL moderate Altered Expression [38]
NEUROD1 OTZQ7QJ2 moderate Altered Expression [41]
NKX2-3 OTF4IL5J moderate Altered Expression [24]
OTP OTS0JN6Y moderate Altered Expression [42]
PPP1R12A OT4AVU95 moderate Biomarker [43]
TEAD2 OTM8YMYT moderate Altered Expression [44]
TMPRSS11A OTB35677 moderate Biomarker [45]
TRAM1 OT3I0H8E moderate Biomarker [37]
FA2H OT8HA13U Strong Altered Expression [46]
IGF2BP3 OTB97VIK Strong Altered Expression [47]
MEN1 OTN6U6V0 Strong Biomarker [31]
NKX2-1 OTCMEJTA Strong Biomarker [36]
NUTM1 OTONYC08 Strong Biomarker [48]
OR51E1 OTB7Q65H Strong Biomarker [49]
RNASET2 OTWY64L7 Strong Biomarker [50]
SOX11 OT4LG7LA Strong Altered Expression [51]
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⏷ Show the Full List of 23 DOT(s)

References

1 2016 FDA drug approvals. Nat Rev Drug Discov. 2017 Feb 2;16(2):73-76.
2 2008 FDA drug approvals. Nat Rev Drug Discov. 2009 Feb;8(2):93-6.
3 2018 FDA drug approvals.Nat Rev Drug Discov. 2019 Feb;18(2):85-89.
4 Octreotide FDA Label
5 FDA Approved Drug Products from FDA Official Website. 2023. Application Number: 761334.
6 ClinicalTrials.gov (NCT04919226) A Prospective, Randomised, Controlled, Open-label, Multicentre Study to Evaluate Efficacy, Safety and Patient-Reported Outcomes of Peptide Receptor Radionuclide Therapy (PRRT) With 177Lu-Edotreotide Compared to Best Standard of Care in Patients With Well-differentiated Aggressive Grade 2 and Grade 3, Somatostatin Receptor-Positive (SSTR+), Neuroendocrine Tumours of GastroEnteric or Pancreatic Origin. U.S.National Institutes of Health.
7 ClinicalTrials.gov (NCT01842165) 177Lutetium-octreotate Treatment Prediction Using Multimodality Imaging in Refractory NETs (LUMEN). U.S. National Institutes of Health.
8 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
9 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
10 Clinical pipeline report, company report or official report of the Pharmaceutical Research and Manufacturers of America (PhRMA)
11 ClinicalTrials.gov (NCT02589821) Phase III Study of Surufatinib in Treating Advanced Pancreatic Neuroendocrine Tumors. U.S. National Institutes of Health.
12 ClinicalTrials.gov (NCT02067988) Holmium-166-radioembolization in NET After Lutetium-177-dotatate; an Efficacy Study (HEPAR_Plus). U.S. National Institutes of Health.
13 ClinicalTrials.gov (NCT03600233) Study of CVM-1118 for Patients With Advanced Neuroendocrine Tumors. U.S. National Institutes of Health.
14 ClinicalTrials.gov (NCT02592707) Study to Evaluate the Safety and Preliminary Efficacy of 177Lu-OPS201 in NETs. U.S. National Institutes of Health.
15 ClinicalTrials.gov (NCT03466216) Phase 1 Study of AlphaMedix in Adult Subjects With SSTR (+) NET. U.S. National Institutes of Health.
16 ClinicalTrials.gov (NCT04702737) A Study of AMG 757 in Participants With Neuroendocrine Prostate Cancer. U.S. National Institutes of Health.
17 URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Target id: 356).
18 URL: http://www.guidetopharmacology.org Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Target id: 359).
19 IGF-1 drives chromogranin A secretion via activation of Arf1 in human neuroendocrine tumour cells.J Cell Mol Med. 2015 May;19(5):948-59. doi: 10.1111/jcmm.12473. Epub 2015 Mar 8.
20 Novel Finding of Paired Box 5 (PAX5) Cytoplasmic Staining in Well-differentiated Rectal Neuroendocrine Tumors (Carcinoids) and Its Diagnostic and Potentially Prognostic Utility.Appl Immunohistochem Mol Morphol. 2019 Jul;27(6):454-460. doi: 10.1097/PAI.0000000000000635.
21 99mTc-MDP Uptake in Diffuse Pleural Recurrences of Pulmonary Neuroendocrine Carcinoma.Clin Nucl Med. 2017 Feb;42(2):133-134. doi: 10.1097/RLU.0000000000001473.
22 Contribution of glutaredoxin-1 to S-glutathionylation of endothelial nitric oxide synthase for mesenteric nitric oxide generation in experimental necrotizing enterocolitis.Transl Res. 2017 Oct;188:92-105. doi: 10.1016/j.trsl.2016.01.004. Epub 2016 Jan 18.
23 Association of neonatal necrotizing enterocolitis with myeloid differentiation-2 and GM2 activator protein genetic polymorphisms.Mol Med Rep. 2015 Jul;12(1):974-80. doi: 10.3892/mmr.2015.3499. Epub 2015 Mar 17.
24 Novel markers for enterochromaffin cells and gastrointestinal neuroendocrine carcinomas.Mod Pathol. 2009 Feb;22(2):261-72. doi: 10.1038/modpathol.2008.174. Epub 2008 Oct 24.
25 Does neutrophil/lymphocyte ratio have good diagnostic value in neonatal necrotizing colitis?.J Matern Fetal Neonatal Med. 2019 Sep;32(18):3026-3033. doi: 10.1080/14767058.2018.1455182. Epub 2018 Mar 27.
26 Clinical value of (99m)Tc-HYNIC-TOC SPECT/CT for the diagnosis primary small cell neuroendocrine carcinomas of the nasal cavity and paranasal sinuses.Clin Imaging. 2018 Nov-Dec;52:365-369. doi: 10.1016/j.clinimag.2018.09.010. Epub 2018 Sep 20.
27 A chemical biology screen identifies a vulnerability of neuroendocrine cancer cells to SQLE inhibition.Nat Commun. 2019 Jan 9;10(1):96. doi: 10.1038/s41467-018-07959-4.
28 Morphologic Patterns and the Correlation With MYD88 L265P, CD79B Mutations in Primary Adrenal Diffuse Large B-Cell Lymphoma.Am J Surg Pathol. 2020 Apr;44(4):444-455. doi: 10.1097/PAS.0000000000001386.
29 Expression of p21Cip1/Waf1 and p27Kip1 in small cell neuroendocrine carcinoma of the uterine cervix.Int J Surg Pathol. 2008 Jan;16(1):11-5. doi: 10.1177/1066896907309574.
30 Delta-like 3 localizes to neuroendocrine cells and plays a pivotal role in gastrointestinal neuroendocrine malignancy.Cancer Sci. 2019 Oct;110(10):3122-3131. doi: 10.1111/cas.14157. Epub 2019 Aug 14.
31 Two well-differentiated pancreatic neuroendocrine tumor mouse models.Cell Death Differ. 2020 Jan;27(1):269-283. doi: 10.1038/s41418-019-0355-0. Epub 2019 Jun 3.
32 Management of Small Bowel Neuroendocrine Tumors.Cancers (Basel). 2019 Sep 18;11(9):1395. doi: 10.3390/cancers11091395.
33 Programmed Death Ligand 1 (PD-L1) Status and Tumor-Infiltrating Lymphocytes in Hot Spots of Primary and Liver Metastases in Prostate Cancer With Neuroendocrine Differentiation.Clin Genitourin Cancer. 2019 Apr;17(2):145-153.e5. doi: 10.1016/j.clgc.2018.12.007. Epub 2018 Dec 21.
34 APOBEC3B High Expression in Gastroenteropancreatic Neuroendocrine Neoplasms and Association With Lymph Metastasis.Appl Immunohistochem Mol Morphol. 2019 Sep;27(8):599-605. doi: 10.1097/PAI.0000000000000695.
35 A case of mixed adenoneuroendocrine carcinoma of the pancreas: Immunohistochemical analysis for histogenesis.Medicine (Baltimore). 2017 Mar;96(9):e6225. doi: 10.1097/MD.0000000000006225.
36 Differentiating Merkel cell carcinoma of lymph nodes without a detectable primary skin tumor from other metastatic neuroendocrine carcinomas: The ELECTHIP criteria.J Am Acad Dermatol. 2018 May;78(5):964-972.e3. doi: 10.1016/j.jaad.2017.11.037. Epub 2017 Nov 24.
37 The landscape of somatic chromosomal copy number aberrations in GEM models of prostate carcinoma.Mol Cancer Res. 2015 Feb;13(2):339-47. doi: 10.1158/1541-7786.MCR-14-0262. Epub 2014 Oct 8.
38 Expression of squamous cell carcinoma markers and adenocarcinoma markers in primary pulmonary neuroendocrine carcinomas.Appl Immunohistochem Mol Morphol. 2013 Jul;21(4):292-7. doi: 10.1097/PAI.0b013e31826fd4f3.
39 Insulinoma-associated Protein 1 (INSM1) in Thoracic Tumors is Less Sensitive but More Specific Compared With Synaptophysin, Chromogranin A, and CD56.Appl Immunohistochem Mol Morphol. 2020 Mar;28(3):237-242. doi: 10.1097/PAI.0000000000000715.
40 The expression of TTF1, CDX2 and ISL1 in 74 poorly differentiated neuroendocrine carcinomas.Ann Diagn Pathol. 2018 Dec;37:30-34. doi: 10.1016/j.anndiagpath.2018.09.005. Epub 2018 Sep 13.
41 The expression of NeuroD and mASH1 in the gastroenteropancreatic neuroendocrine tumors.Mod Pathol. 2008 Nov;21(11):1363-70. doi: 10.1038/modpathol.2008.121. Epub 2008 Jun 27.
42 Expression of Insulinoma-Associated Protein 1 (INSM1) and Orthopedia Homeobox (OTP) in Tumors with Neuroendocrine Differentiation at Rare Sites.Endocr Pathol. 2019 Mar;30(1):35-42. doi: 10.1007/s12022-018-9559-y.
43 A comparison of adult rhabdomyosarcoma and high-grade neuroendocrine carcinoma of the urinary bladder reveals novel PPP1R12A fusions in rhabdomyosarcoma.Hum Pathol. 2019 Jun;88:48-59. doi: 10.1016/j.humpath.2019.03.007. Epub 2019 Apr 1.
44 SOX4, SOX11 and PAX6 mRNA expression was identified as a (prognostic) marker for the aggressiveness of neuroendocrine tumors of the lung by using next-generation expression analysis (NanoString).Future Oncol. 2015;11(7):1027-36. doi: 10.2217/fon.15.18.
45 ECRG1, a novel esophageal gene, cloned and identified from human esophagus and its inhibition effect on tumors.Carcinogenesis. 2008 Jan;29(1):157-60. doi: 10.1093/carcin/bgm203. Epub 2007 Oct 29.
46 High FA2H and UGT8 transcript levels predict hydroxylated hexosylceramide accumulation in lung adenocarcinoma.J Lipid Res. 2019 Oct;60(10):1776-1786. doi: 10.1194/jlr.M093955. Epub 2019 Aug 13.
47 Merkel cell carcinoma expresses K homology domain-containing protein overexpressed in cancer similar to other high-grade neuroendocrine carcinomas.Hum Pathol. 2009 Feb;40(2):238-43. doi: 10.1016/j.humpath.2008.07.009. Epub 2008 Oct 5.
48 Cytologic findings of NUT midline carcinoma in the hilum of the lung.Diagn Cytopathol. 2015 Sep;43(9):739-42. doi: 10.1002/dc.23291. Epub 2015 Jul 3.
49 Olfactory receptor 51E1 protein as a potential novel tissue biomarker for small intestine neuroendocrine carcinomas.Eur J Endocrinol. 2013 Jan 17;168(2):253-61. doi: 10.1530/EJE-12-0814. Print 2013 Feb.
50 New insights into hypoxia-related mechanisms involved in different microvascular patterns of bronchopulmonary carcinoids and poorly differentiated neuroendocrine carcinomas. Role of ribonuclease T2 (RNASET2) and HIF-1.Hum Pathol. 2018 Sep;79:66-76. doi: 10.1016/j.humpath.2018.04.028. Epub 2018 May 12.
51 SOX11: a potentially useful marker in surgical pathology: a systematic analysis of SOX11 expression in epithelial and non-epithelial tumours.Histopathology. 2019 Feb;74(3):391-405. doi: 10.1111/his.13757. Epub 2018 Dec 2.