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Intestinal Neuroendocrine Carcinoma

MedGen UID:
474163
•Concept ID:
C3272530
•
Neoplastic Process
Synonyms: Intestinal High Grade Neuroendocrine Carcinoma; Intestinal NEC; Intestinal NEC G3; Intestinal Poorly Differentiated Endocrine Carcinoma

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the small or large intestine. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. [from NCI]

Professional guidelines

PubMed

An Organoid Biobank of Neuroendocrine Neoplasms Enables Genotype-Phenotype Mapping.
Kawasaki K, Toshimitsu K, Matano M, Fujita M, Fujii M, Togasaki K, Ebisudani T, Shimokawa M, Takano A, Takahashi S, Ohta Y, Nanki K, Igarashi R, Ishimaru K, Ishida H, Sukawa Y, Sugimoto S, Saito Y, Maejima K, Sasagawa S, Lee H, Kim HG, Ha K, Hamamoto J, Fukunaga K, Maekawa A, Tanabe M, Ishihara S, Hamamoto Y, Yasuda H, Sekine S, Kudo A, Kitagawa Y, Kanai T, Nakagawa H, Sato T
Cell 2020 Nov 25;183(5):1420-1435.e21. Epub 2020 Nov 6 doi: 10.1016/j.cell.2020.10.023. PMID: 33159857
Current Treatment Options in Gastroenteropancreatic Neuroendocrine Carcinoma.
Thomas KEH, Voros BA, Boudreaux JP, Thiagarajan R, Woltering EA, Ramirez RA
Oncologist 2019 Aug;24(8):1076-1088. Epub 2019 Jan 11 doi: 10.1634/theoncologist.2018-0604. PMID: 30635447Free PMC Article
ENETS Consensus Guidelines for High-Grade Gastroenteropancreatic Neuroendocrine Tumors and Neuroendocrine Carcinomas.
Garcia-Carbonero R, Sorbye H, Baudin E, Raymond E, Wiedenmann B, Niederle B, Sedlackova E, Toumpanakis C, Anlauf M, Cwikla JB, Caplin M, O'Toole D, Perren A; Vienna Consensus Conference participants
Neuroendocrinology 2016;103(2):186-94. Epub 2016 Jan 5 doi: 10.1159/000443172. PMID: 26731334

Recent clinical studies

Etiology

Neoplasms of the Appendix.
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Challenging differential diagnoses in small biopsies from the sinonasal tract.
Israel AK, Cracolici V, Griffith CC
Semin Diagn Pathol 2023 Sep;40(5):321-332. Epub 2023 Apr 14 doi: 10.1053/j.semdp.2023.04.011. PMID: 37085435
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Types of Gastric Carcinomas.
Waldum HL, Fossmark R
Int J Mol Sci 2018 Dec 18;19(12) doi: 10.3390/ijms19124109. PMID: 30567376Free PMC Article

Diagnosis

Neoplasms of the Appendix.
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Types of Gastric Carcinomas.
Waldum HL, Fossmark R
Int J Mol Sci 2018 Dec 18;19(12) doi: 10.3390/ijms19124109. PMID: 30567376Free PMC Article
ENETS Consensus Guidelines for High-Grade Gastroenteropancreatic Neuroendocrine Tumors and Neuroendocrine Carcinomas.
Garcia-Carbonero R, Sorbye H, Baudin E, Raymond E, Wiedenmann B, Niederle B, Sedlackova E, Toumpanakis C, Anlauf M, Cwikla JB, Caplin M, O'Toole D, Perren A; Vienna Consensus Conference participants
Neuroendocrinology 2016;103(2):186-94. Epub 2016 Jan 5 doi: 10.1159/000443172. PMID: 26731334

Therapy

Neoplasms of the Appendix.
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Models of Gastroenteropancreatic Neuroendocrine Neoplasms: Current Status and Future Directions.
Detjen K, Hammerich L, Özdirik B, Demir M, Wiedenmann B, Tacke F, Jann H, Roderburg C
Neuroendocrinology 2021;111(3):217-236. Epub 2020 Jul 2 doi: 10.1159/000509864. PMID: 32615560
PRRT in high-grade gastroenteropancreatic neuroendocrine neoplasms (WHO G3).
Sorbye H, Kong G, Grozinsky-Glasberg S
Endocr Relat Cancer 2020 Mar;27(3):R67-R77. doi: 10.1530/ERC-19-0400. PMID: 31846429
RET fusions in solid tumors.
Li AY, McCusker MG, Russo A, Scilla KA, Gittens A, Arensmeyer K, Mehra R, Adamo V, Rolfo C
Cancer Treat Rev 2019 Dec;81:101911. Epub 2019 Oct 30 doi: 10.1016/j.ctrv.2019.101911. PMID: 31715421
Current Treatment Options in Gastroenteropancreatic Neuroendocrine Carcinoma.
Thomas KEH, Voros BA, Boudreaux JP, Thiagarajan R, Woltering EA, Ramirez RA
Oncologist 2019 Aug;24(8):1076-1088. Epub 2019 Jan 11 doi: 10.1634/theoncologist.2018-0604. PMID: 30635447Free PMC Article

Prognosis

Genomic characterization reveals distinct mutation landscapes and therapeutic implications in neuroendocrine carcinomas of the gastrointestinal tract.
Wu H, Yu Z, Liu Y, Guo L, Teng L, Guo L, Liang L, Wang J, Gao J, Li R, Yang L, Nie X, Su D, Liang Z
Cancer Commun (Lond) 2022 Dec;42(12):1367-1386. Epub 2022 Oct 20 doi: 10.1002/cac2.12372. PMID: 36264285Free PMC Article
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Types of Gastric Carcinomas.
Waldum HL, Fossmark R
Int J Mol Sci 2018 Dec 18;19(12) doi: 10.3390/ijms19124109. PMID: 30567376Free PMC Article

Clinical prediction guides

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Clinical and pathological profile of gastric neuroendocrine tumors.
Sekar A, Vaiphei K
Indian J Pathol Microbiol 2022 Jul-Sep;65(3):551-557. doi: 10.4103/ijpm.ijpm_824_21. PMID: 35900481
Models of Gastroenteropancreatic Neuroendocrine Neoplasms: Current Status and Future Directions.
Detjen K, Hammerich L, Özdirik B, Demir M, Wiedenmann B, Tacke F, Jann H, Roderburg C
Neuroendocrinology 2021;111(3):217-236. Epub 2020 Jul 2 doi: 10.1159/000509864. PMID: 32615560
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
VIPoma syndrome.
Krejs GJ
Am J Med 1987 May 29;82(5B):37-48. doi: 10.1016/0002-9343(87)90425-6. PMID: 3035922

Recent systematic reviews

Management Strategies and Outcomes for Small Intestinal Neuroendocrine Tumours with Involvement of the Superior Mesenteric Vessels: A Systematic Review.
Kmiotek E, Lakda S, Borakati A, Ogunbiyi O, Mandair D, Caplin M, Toumpanakis C, Mirnezami R
Curr Oncol 2023 Oct 18;30(10):9192-9204. doi: 10.3390/curroncol30100664. PMID: 37887564Free PMC Article
Management and outcomes of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A systematic review and pooled analysis of 56 patients.
Ziogas IA, Rallis KS, Tasoudis PT, Moris D, Schulick RD, Del Chiaro M
Eur J Surg Oncol 2023 Apr;49(4):682-687. Epub 2023 Jan 9 doi: 10.1016/j.ejso.2023.01.005. PMID: 36646615
Somatostatinoma of the Ampulla of Vater: A Systematic Review.
Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044
Neuroendocrine neoplasms of gastrointestinal tract and secondary primary synchronous tumors: A systematic review of case reports. Casualty or causality?
Parra-Medina R, Moreno-Lucero P, Jimenez-Moreno J, Parra-Morales AM, Romero-Rojas A
PLoS One 2019;14(5):e0216647. Epub 2019 May 14 doi: 10.1371/journal.pone.0216647. PMID: 31086397Free PMC Article
Liver transplantation for hepatic metastases of neuroendocrine pancreatic tumors: a survival-based analysis.
Máthé Z, Tagkalos E, Paul A, Molmenti EP, Kóbori L, Fouzas I, Beckebaum S, Sotiropoulos GC
Transplantation 2011 Mar 15;91(5):575-82. doi: 10.1097/TP.0b013e3182081312. PMID: 21200365

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