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Small Intestinal Neuroendocrine Carcinoma

MedGen UID:
474164
•Concept ID:
C3272531
•
Neoplastic Process
Synonyms: Small Intestinal High Grade Neuroendocrine Carcinoma; Small Intestinal NEC; Small Intestinal Poorly Differentiated Endocrine Carcinoma

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the small 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

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
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
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

Recent clinical studies

Etiology

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
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
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Primary small-cell neuroendocrine carcinoma of the bladder: Case report and literature review.
Olivieri V, Fortunati V, Bellei L, Massarelli M, Ruggiero G, Abate D, Serra N, Griffa D, Forte F, Corongiu E
Arch Ital Urol Androl 2020 Oct 2;92(3) doi: 10.4081/aiua.2020.3.211. PMID: 33016048
Composite intestinal adenoma-microcarcinoid.
Lin J, Goldblum JR, Bennett AE, Bronner MP, Liu X
Am J Surg Pathol 2012 Feb;36(2):292-5. doi: 10.1097/PAS.0b013e318236e4d2. PMID: 22082602

Diagnosis

Multi-omic dataset of patient-derived tumor organoids of neuroendocrine neoplasms.
Alcala N, Voegele C, Mangiante L, Sexton-Oates A, Clevers H, Fernandez-Cuesta L, Dayton TL, Foll M
Gigascience 2024 Jan 2;13 doi: 10.1093/gigascience/giae008. PMID: 38451475Free PMC Article
Expression of G protein-coupled receptor GPR19 in normal and neoplastic human tissues.
Gerlach L, Beyer AL, Kaemmerer D, Sänger J, Evert K, Schulz S, Lupp A
Sci Rep 2023 Nov 3;13(1):18993. doi: 10.1038/s41598-023-46395-3. PMID: 37923782Free 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
Incidence, survival and stage at diagnosis of small intestinal neuroendocrine tumours in Queensland, Australia, 2001-2015.
Wyld D, Moore J, Tran N, Youl P
Asia Pac J Clin Oncol 2021 Aug;17(4):350-358. Epub 2021 Feb 10 doi: 10.1111/ajco.13503. PMID: 33567164
Diagnostic strategy with a solid pancreatic mass.
Guarneri G, Gasparini G, Crippa S, Andreasi V, Falconi M
Presse Med 2019 Mar;48(3 Pt 2):e125-e145. Epub 2019 Mar 14 doi: 10.1016/j.lpm.2019.02.026. PMID: 30878333

Therapy

Multi-omic dataset of patient-derived tumor organoids of neuroendocrine neoplasms.
Alcala N, Voegele C, Mangiante L, Sexton-Oates A, Clevers H, Fernandez-Cuesta L, Dayton TL, Foll M
Gigascience 2024 Jan 2;13 doi: 10.1093/gigascience/giae008. PMID: 38451475Free PMC Article
Surgical Principles in the Management of Small Bowel Neuroendocrine Tumors.
Gangi A, Anaya DA
Curr Treat Options Oncol 2020 Aug 29;21(11):88. doi: 10.1007/s11864-020-00784-2. PMID: 32862334
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
Heterogeneity of grade 3 gastroenteropancreatic neuroendocrine carcinomas: New insights and treatment implications.
Fazio N, Milione M
Cancer Treat Rev 2016 Nov;50:61-67. Epub 2016 Aug 28 doi: 10.1016/j.ctrv.2016.08.006. PMID: 27636009

Prognosis

Neuroendocrine tumor G3 of bronchopulmonary origin and its classification.
Kasajima A, Klöppel G
Pathol Int 2022 Oct;72(10):488-495. Epub 2022 Aug 19 doi: 10.1111/pin.13266. PMID: 35983917
The molecular characteristics of high-grade gastroenteropancreatic neuroendocrine neoplasms.
Venizelos A, Elvebakken H, Perren A, Nikolaienko O, Deng W, Lothe IMB, Couvelard A, Hjortland GO, Sundlöv A, Svensson J, Garresori H, Kersten C, Hofsli E, Detlefsen S, Krogh M, Sorbye H, Knappskog S
Endocr Relat Cancer 2021 Nov 11;29(1):1-14. doi: 10.1530/ERC-21-0152. PMID: 34647903Free 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
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
Composite intestinal adenoma-microcarcinoid.
Lin J, Goldblum JR, Bennett AE, Bronner MP, Liu X
Am J Surg Pathol 2012 Feb;36(2):292-5. doi: 10.1097/PAS.0b013e318236e4d2. PMID: 22082602

Clinical prediction guides

Expression of G protein-coupled receptor GPR19 in normal and neoplastic human tissues.
Gerlach L, Beyer AL, Kaemmerer D, Sänger J, Evert K, Schulz S, Lupp A
Sci Rep 2023 Nov 3;13(1):18993. doi: 10.1038/s41598-023-46395-3. PMID: 37923782Free PMC Article
Islet-1 Is Differentially Expressed Among Neuroendocrine and Non-Neuroendocrine Tumors and Its Potential Diagnostic Implication.
Xiang Y, Malik F, Zhang PJ
Int J Surg Pathol 2023 Oct;31(7):1294-1301. Epub 2023 Feb 21 doi: 10.1177/10668969231157319. PMID: 37796752
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
Small-cell neuroendocrine carcinoma of the ileum: case report and literature review.
Lee JE, Hong SH, Jung HI, Son MW, Ahn TS, Han SW, Cho JH
BMC Surg 2019 Sep 12;19(1):135. doi: 10.1186/s12893-019-0591-8. PMID: 31510991Free PMC Article
Pathology of gastrointestinal disorders.
Rindi G, Inzani F, Solcia E
Endocrinol Metab Clin North Am 2010 Dec;39(4):713-27. doi: 10.1016/j.ecl.2010.08.009. PMID: 21095540

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

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