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Neuroendocrine carcinoma

MedGen UID:
104919
•Concept ID:
C0206695
•
Neoplastic Process
Synonyms: Carcinoma, Neuroendocrine; Carcinomas, Neuroendocrine; Neuroendocrine Carcinoma; Neuroendocrine Carcinomas
SNOMED CT: Neuroendocrine carcinoma (253000007); Neuroendocrine carcinoma (55937004)
 
Monarch Initiative: MONDO:0002120

Definition

A usually aggressive carcinoma composed of malignant cells exhibiting neuroendocrine differentiation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNeuroendocrine carcinoma

Professional guidelines

PubMed

International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors.
Kuan EC, Wang EW, Adappa ND, Beswick DM, London NR Jr, Su SY, Wang MB, Abuzeid WM, Alexiev B, Alt JA, Antognoni P, Alonso-Basanta M, Batra PS, Bhayani M, Bell D, Bernal-Sprekelsen M, Betz CS, Blay JY, Bleier BS, Bonilla-Velez J, Callejas C, Carrau RL, Casiano RR, Castelnuovo P, Chandra RK, Chatzinakis V, Chen SB, Chiu AG, Choby G, Chowdhury NI, Citardi MJ, Cohen MA, Dagan R, Dalfino G, Dallan I, Dassi CS, de Almeida J, Dei Tos AP, DelGaudio JM, Ebert CS, El-Sayed IH, Eloy JA, Evans JJ, Fang CH, Farrell NF, Ferrari M, Fischbein N, Folbe A, Fokkens WJ, Fox MG, Lund VJ, Gallia GL, Gardner PA, Geltzeiler M, Georgalas C, Getz AE, Govindaraj S, Gray ST, Grayson JW, Gross BA, Grube JG, Guo R, Ha PK, Halderman AA, Hanna EY, Harvey RJ, Hernandez SC, Holtzman AL, Hopkins C, Huang Z, Huang Z, Humphreys IM, Hwang PH, Iloreta AM, Ishii M, Ivan ME, Jafari A, Kennedy DW, Khan M, Kimple AJ, Kingdom TT, Knisely A, Kuo YJ, Lal D, Lamarre ED, Lan MY, Le H, Lechner M, Lee NY, Lee JK, Lee VH, Levine CG, Lin JC, Lin DT, Lobo BC, Locke T, Luong AU, Magliocca KR, Markovic SN, Matnjani G, McKean EL, Meço C, Mendenhall WM, Michel L, Na'ara S, Nicolai P, Nuss DW, Nyquist GG, Oakley GM, Omura K, Orlandi RR, Otori N, Papagiannopoulos P, Patel ZM, Pfister DG, Phan J, Psaltis AJ, Rabinowitz MR, Ramanathan M Jr, Rimmer R, Rosen MR, Sanusi O, Sargi ZB, Schafhausen P, Schlosser RJ, Sedaghat AR, Senior BA, Shrivastava R, Sindwani R, Smith TL, Smith KA, Snyderman CH, Solares CA, Sreenath SB, Stamm A, Stölzel K, Sumer B, Surda P, Tajudeen BA, Thompson LDR, Thorp BD, Tong CCL, Tsang RK, Turner JH, Turri-Zanoni M, Udager AM, van Zele T, VanKoevering K, Welch KC, Wise SK, Witterick IJ, Won TB, Wong SN, Woodworth BA, Wormald PJ, Yao WC, Yeh CF, Zhou B, Palmer JN
Int Forum Allergy Rhinol 2024 Feb;14(2):149-608. Epub 2024 Jan 2 doi: 10.1002/alr.23262. PMID: 37658764
Merkel cell carcinoma: An updated review of pathogenesis, diagnosis, and treatment options.
Hernandez LE, Mohsin N, Yaghi M, Frech FS, Dreyfuss I, Nouri K
Dermatol Ther 2022 Mar;35(3):e15292. Epub 2022 Jan 5 doi: 10.1111/dth.15292. PMID: 34967084
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190

Recent clinical studies

Etiology

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma.
Sorbye H, Grande E, Pavel M, Tesselaar M, Fazio N, Reed NS, Knigge U, Christ E, Ambrosini V, Couvelard A, Tiensuu Janson E
J Neuroendocrinol 2023 Mar;35(3):e13249. Epub 2023 Mar 16 doi: 10.1111/jne.13249. PMID: 36924180
Integrated Genomic and Clinicopathologic Approach Distinguishes Pancreatic Grade 3 Neuroendocrine Tumor From Neuroendocrine Carcinoma and Identifies a Subset With Molecular Overlap.
Umetsu SE, Kakar S, Basturk O, Kim GE, Chatterjee D, Wen KW, Hale G, Shafizadeh N, Cho SJ, Whitman J, Gill RM, Jones KD, Navale P, Bergsland E, Klimstra D, Joseph NM
Mod Pathol 2023 Mar;36(3):100065. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100065. PMID: 36788102
Neuroendocrine neoplasms of the esophagus and stomach.
Mastracci L, Rindi G, Grillo F, Solcia E, Campora M, Fassan M, Parente P, Vanoli A, La Rosa S
Pathologica 2021 Feb;113(1):5-11. doi: 10.32074/1591-951X-229. PMID: 33686305Free PMC Article
Appendiceal cancer : a review of the literature.
Van de Moortele M, De Hertogh G, Sagaert X, Van Cutsem E
Acta Gastroenterol Belg 2020 Jul-Sep;83(3):441-448. PMID: 33094592
Nothing But NET: A Review of Neuroendocrine Tumors and Carcinomas.
Oronsky B, Ma PC, Morgensztern D, Carter CA
Neoplasia 2017 Dec;19(12):991-1002. Epub 2017 Nov 5 doi: 10.1016/j.neo.2017.09.002. PMID: 29091800Free PMC Article

Diagnosis

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma.
Sorbye H, Grande E, Pavel M, Tesselaar M, Fazio N, Reed NS, Knigge U, Christ E, Ambrosini V, Couvelard A, Tiensuu Janson E
J Neuroendocrinol 2023 Mar;35(3):e13249. Epub 2023 Mar 16 doi: 10.1111/jne.13249. PMID: 36924180
Pulmonary Combined Large Cell Neuroendocrine Carcinoma.
Li M, Yang L, Lu H
Pathol Oncol Res 2022;28:1610747. Epub 2022 Nov 23 doi: 10.3389/pore.2022.1610747. PMID: 36507119Free PMC Article
Neuroendocrine Tumors of the Thyroid and Their Mimics.
Livolsi VA
Endocr Pathol 2021 Jun;32(2):211-221. Epub 2021 Mar 9 doi: 10.1007/s12022-021-09672-3. PMID: 33687689
Diagnosis and Management of Gastrointestinal Neuroendocrine Neoplasms.
Gonzalez RS
Surg Pathol Clin 2020 Sep;13(3):377-397. doi: 10.1016/j.path.2020.04.002. PMID: 32773190
Pulmonary Neuroendocrine Tumors.
Borczuk AC
Surg Pathol Clin 2020 Mar;13(1):35-55. Epub 2019 Nov 29 doi: 10.1016/j.path.2019.10.002. PMID: 32005434

Therapy

European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma.
Sorbye H, Grande E, Pavel M, Tesselaar M, Fazio N, Reed NS, Knigge U, Christ E, Ambrosini V, Couvelard A, Tiensuu Janson E
J Neuroendocrinol 2023 Mar;35(3):e13249. Epub 2023 Mar 16 doi: 10.1111/jne.13249. PMID: 36924180
Effectiveness of Etoposide and Cisplatin vs Irinotecan and Cisplatin Therapy for Patients With Advanced Neuroendocrine Carcinoma of the Digestive System: The TOPIC-NEC Phase 3 Randomized Clinical Trial.
Morizane C, Machida N, Honma Y, Okusaka T, Boku N, Kato K, Nomura S, Hiraoka N, Sekine S, Taniguchi H, Okano N, Yamaguchi K, Sato T, Ikeda M, Mizuno N, Ozaka M, Kataoka T, Ueno M, Kitagawa Y, Terashima M, Furuse J; Japan Clinical Oncology Group (JCOG)
JAMA Oncol 2022 Oct 1;8(10):1447-1455. doi: 10.1001/jamaoncol.2022.3395. PMID: 35980649Free PMC Article
Phase I trial of the DLL3/CD3 bispecific T-cell engager BI 764532 in DLL3-positive small-cell lung cancer and neuroendocrine carcinomas.
Wermke M, Felip E, Gambardella V, Kuboki Y, Morgensztern D, Hamed ZO, Liu M, Studeny M, Owonikoko TK
Future Oncol 2022 Aug;18(24):2639-2649. Epub 2022 Jul 11 doi: 10.2217/fon-2022-0196. PMID: 35815644
Appendiceal cancer : a review of the literature.
Van de Moortele M, De Hertogh G, Sagaert X, Van Cutsem E
Acta Gastroenterol Belg 2020 Jul-Sep;83(3):441-448. PMID: 33094592
Neuroendocrine carcinoma of the cervix: a systematic review of the literature.
Tempfer CB, Tischoff I, Dogan A, Hilal Z, Schultheis B, Kern P, Rezniczek GA
BMC Cancer 2018 May 4;18(1):530. doi: 10.1186/s12885-018-4447-x. PMID: 29728073Free PMC Article

Prognosis

An update on the diagnosis of gastroenteropancreatic neuroendocrine neoplasms.
Fang JM, Li J, Shi J
World J Gastroenterol 2022 Mar 14;28(10):1009-1023. doi: 10.3748/wjg.v28.i10.1009. PMID: 35431496Free PMC Article
Epidemiology, Incidence, and Prevalence of Neuroendocrine Neoplasms: Are There Global Differences?
Das S, Dasari A
Curr Oncol Rep 2021 Mar 14;23(4):43. doi: 10.1007/s11912-021-01029-7. PMID: 33719003Free PMC Article
Merkel cell carcinoma: A review.
Walsh NM, Cerroni L
J Cutan Pathol 2021 Mar;48(3):411-421. Epub 2020 Dec 3 doi: 10.1111/cup.13910. PMID: 33128463
Sinonasal Undifferentiated Carcinoma.
Abdelmeguid AS, Bell D, Hanna EY
Curr Oncol Rep 2019 Feb 26;21(3):26. doi: 10.1007/s11912-019-0776-4. PMID: 30806835
Neuroendocrine carcinoma of the cervix: a systematic review of the literature.
Tempfer CB, Tischoff I, Dogan A, Hilal Z, Schultheis B, Kern P, Rezniczek GA
BMC Cancer 2018 May 4;18(1):530. doi: 10.1186/s12885-018-4447-x. PMID: 29728073Free PMC Article

Clinical prediction guides

Narrative Review of Immunotherapy in Gastroentero-Pancreatic Neuroendocrine Neoplasms.
Kaur J, Vijayvergia N
Curr Oncol 2023 Sep 21;30(9):8653-8664. doi: 10.3390/curroncol30090627. PMID: 37754542Free PMC Article
European Neuroendocrine Tumor Society (ENETS) 2023 guidance paper for digestive neuroendocrine carcinoma.
Sorbye H, Grande E, Pavel M, Tesselaar M, Fazio N, Reed NS, Knigge U, Christ E, Ambrosini V, Couvelard A, Tiensuu Janson E
J Neuroendocrinol 2023 Mar;35(3):e13249. Epub 2023 Mar 16 doi: 10.1111/jne.13249. PMID: 36924180
Integrated Genomic and Clinicopathologic Approach Distinguishes Pancreatic Grade 3 Neuroendocrine Tumor From Neuroendocrine Carcinoma and Identifies a Subset With Molecular Overlap.
Umetsu SE, Kakar S, Basturk O, Kim GE, Chatterjee D, Wen KW, Hale G, Shafizadeh N, Cho SJ, Whitman J, Gill RM, Jones KD, Navale P, Bergsland E, Klimstra D, Joseph NM
Mod Pathol 2023 Mar;36(3):100065. Epub 2023 Jan 10 doi: 10.1016/j.modpat.2022.100065. PMID: 36788102
International Medullary Thyroid Carcinoma Grading System: A Validated Grading System for Medullary Thyroid Carcinoma.
Xu B, Fuchs TL, Ahmadi S, Alghamdi M, Alzumaili B, Bani MA, Baudin E, Chou A, De Leo A, Fagin JA, Ganly I, Glover A, Hartl D, Kanaan C, Khneisser P, Najdawi F, Nigam A, Papachristos A, Repaci A, Spanheimer PM, Solaroli E, Untch BR, Barletta JA, Tallini G, Al Ghuzlan A, Gill AJ, Ghossein RA
J Clin Oncol 2022 Jan 1;40(1):96-104. Epub 2021 Nov 3 doi: 10.1200/JCO.21.01329. PMID: 34731032Free PMC Article
Immunohistochemistry in the diagnosis and classification of neuroendocrine neoplasms: what can brown do for you?
Bellizzi AM
Hum Pathol 2020 Feb;96:8-33. Epub 2019 Dec 17 doi: 10.1016/j.humpath.2019.12.002. PMID: 31857137Free PMC Article

Recent systematic reviews

Systematic review of neuroendocrine carcinomas of the oropharynx.
Froehlich MH, Shih MC, Shehee L, Kompelli AR, Aylward A, Nguyen SA, Kaczmar JM, Nathan CA, Day T, Neskey DM
Head Neck 2022 Jul;44(7):1725-1736. Epub 2022 Apr 18 doi: 10.1002/hed.27055. PMID: 35437851
Oral and Maxillofacial Neuroendocrine Carcinoma: A Systematic Review.
Schuch LF, Schmidt TR, de Oliveira Zigmundo G, Kirschnick LB, Silveira FM, Martins MAT, Carlos R, Dos Santos JN, Fonseca FP, Vargas PA, Wagner VP, Martins MD
Head Neck Pathol 2022 Jun;16(2):525-537. Epub 2021 Dec 6 doi: 10.1007/s12105-021-01398-2. PMID: 34870796Free PMC Article
Commonwealth Neuroendocrine Tumour Research Collaboration and the North American Neuroendocrine Tumor Society Guidelines for the Diagnosis and Management of Patients With Lung Neuroendocrine Tumors: An International Collaborative Endorsement and Update of the 2015 European Neuroendocrine Tumor Society Expert Consensus Guidelines.
Singh S, Bergsland EK, Card CM, Hope TA, Kunz PL, Laidley DT, Lawrence B, Leyden S, Metz DC, Michael M, Modahl LE, Myrehaug S, Padda SK, Pommier RF, Ramirez RA, Soulen M, Strosberg J, Sung A, Thawer A, Wei B, Xu B, Segelov E
J Thorac Oncol 2020 Oct;15(10):1577-1598. Epub 2020 Jul 11 doi: 10.1016/j.jtho.2020.06.021. PMID: 32663527
Neuroendocrine carcinoma of the cervix: a systematic review of the literature.
Tempfer CB, Tischoff I, Dogan A, Hilal Z, Schultheis B, Kern P, Rezniczek GA
BMC Cancer 2018 May 4;18(1):530. doi: 10.1186/s12885-018-4447-x. PMID: 29728073Free PMC Article
Sinonasal small cell neuroendocrine carcinoma: a systematic review of 80 patients.
Rivero A, Liang J
Int Forum Allergy Rhinol 2016 Jul;6(7):744-51. Epub 2016 Feb 16 doi: 10.1002/alr.21734. PMID: 26880574

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