U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Small Intestinal Large Cell Neuroendocrine Carcinoma

MedGen UID:
474165
•Concept ID:
C3272532
•
Neoplastic Process

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the small intestine. It is characterized by the presence of malignant large cells. [from NCI]

Professional guidelines

PubMed

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
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

Recent clinical studies

Etiology

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
High-Grade Gastrointestinal Neuroendocrine Carcinoma Management and Outcomes: A National Cancer Database Study.
Alese OB, Jiang R, Shaib W, Wu C, Akce M, Behera M, El-Rayes BF
Oncologist 2019 Jul;24(7):911-920. Epub 2018 Nov 27 doi: 10.1634/theoncologist.2018-0382. PMID: 30482824Free 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
The epidemiology and pathogenesis of neoplasia in the small intestine.
Schottenfeld D, Beebe-Dimmer JL, Vigneau FD
Ann Epidemiol 2009 Jan;19(1):58-69. doi: 10.1016/j.annepidem.2008.10.004. PMID: 19064190Free PMC Article

Diagnosis

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
Outcomes of small-cell versus large-cell gastroenteropancreatic neuroendocrine carcinomas: A population-based study.
Abdel-Rahman O, Fazio N
J Neuroendocrinol 2021 May;33(5):e12971. Epub 2021 Apr 19 doi: 10.1111/jne.12971. PMID: 33870570
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
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
Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review.
Acosta AM, Wiley EL
Arch Pathol Lab Med 2016 Oct;140(10):1157-62. doi: 10.5858/arpa.2015-0102-RS. PMID: 27684986

Therapy

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
New model for gastroenteropancreatic large-cell neuroendocrine carcinoma: establishment of two clinically relevant cell lines.
Krieg A, Mersch S, Boeck I, Dizdar L, Weihe E, Hilal Z, Krausch M, Möhlendick B, Topp SA, Piekorz RP, Huckenbeck W, Stoecklein NH, Anlauf M, Knoefel WT
PLoS One 2014;9(2):e88713. Epub 2014 Feb 14 doi: 10.1371/journal.pone.0088713. PMID: 24551139Free PMC Article
Are G3 ENETS neuroendocrine neoplasms heterogeneous?
Vélayoudom-Céphise FL, Duvillard P, Foucan L, Hadoux J, Chougnet CN, Leboulleux S, Malka D, Guigay J, Goere D, Debaere T, Caramella C, Schlumberger M, Planchard D, Elias D, Ducreux M, Scoazec JY, Baudin E
Endocr Relat Cancer 2013 Oct;20(5):649-57. Epub 2013 Aug 19 doi: 10.1530/ERC-13-0027. PMID: 23845449
Peptide receptors in neuroendocrine tumors of the lung as potential tools for radionuclide diagnosis and therapy.
Pelosi G, Volante M, Papotti M, Sonzogni A, Masullo M, Viale G
Q J Nucl Med Mol Imaging 2006 Dec;50(4):272-87. PMID: 17043625

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
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
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
Genetics and Epigenetics of Gastroenteropancreatic Neuroendocrine Neoplasms.
Mafficini A, Scarpa A
Endocr Rev 2019 Apr 1;40(2):506-536. doi: 10.1210/er.2018-00160. PMID: 30657883Free PMC Article
Large cell neuroendocrine carcinoma of the ampulla of Vater.
Beggs RE, Kelly ME, Eltayeb O, Crotty P, McDermott R, Ridgway PF
JOP 2012 Sep 10;13(5):470-5. doi: 10.6092/1590-8577/821. PMID: 22964952

Clinical prediction guides

Pitx2 is a useful marker of midgut-derived neuroendocrine tumours - an immunohistochemical study of 224 cases.
Soukup J, Manethova M, Faistova H, Krbal L, Vitovcova B, Hornychova H, Drugda J, Cesak T, Netuka D, Gabalec F, Ryska A
Histopathology 2022 Dec;81(6):799-807. Epub 2022 Sep 22 doi: 10.1111/his.14789. PMID: 36089904
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
Lung and digestive neuroendocrine neoplasms. From WHO classification to biomarker screening: Which perspectives?
Scoazec JY
Ann Endocrinol (Paris) 2019 Jun;80(3):163-165. Epub 2019 Apr 11 doi: 10.1016/j.ando.2019.04.011. PMID: 31064659
A case of small intestinal neuroendocrine carcinoma diagnosed using double-balloon endoscopy with long-term survival.
Kawano S, Miyashima Y, Miyabe Y, Kawai Y, Murata T, Uda M, Inokuchi T, Okada H
Clin J Gastroenterol 2018 Jun;11(3):240-244. Epub 2018 Feb 15 doi: 10.1007/s12328-018-0834-8. PMID: 29450787
New model for gastroenteropancreatic large-cell neuroendocrine carcinoma: establishment of two clinically relevant cell lines.
Krieg A, Mersch S, Boeck I, Dizdar L, Weihe E, Hilal Z, Krausch M, Möhlendick B, Topp SA, Piekorz RP, Huckenbeck W, Stoecklein NH, Anlauf M, Knoefel WT
PLoS One 2014;9(2):e88713. Epub 2014 Feb 14 doi: 10.1371/journal.pone.0088713. PMID: 24551139Free PMC Article

Recent systematic reviews

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

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...