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Pancreatic large cell neuroendocrine carcinoma

MedGen UID:
459465
•Concept ID:
C2987239
•
Neoplastic Process
Synonyms: large cell neuroendocrine carcinoma of pancreas; pancreas large cell neuroendocrine carcinoma; Pancreatic Large Cell NEC; pancreatic large cell NEC; Pancreatic Large Cell NEC G3; pancreatic large cell NEC G3; Pancreatic Large Cell Neuroendocrine Carcinoma; pancreatic large cell neuroendocrine carcinoma
 
Monarch Initiative: MONDO:0006347

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPancreatic large cell neuroendocrine carcinoma

Professional guidelines

PubMed

Management of Localized Small- and Large-Cell Pancreatic Neuroendocrine Carcinoma in the National Cancer Database.
Sugawara T, Rodriguez Franco S, Franklin O, Kirsch MJ, Colborn KL, Del Chiaro M, Schulick RD
J Am Coll Surg 2023 Sep 1;237(3):515-524. Epub 2023 May 5 doi: 10.1097/XCS.0000000000000735. PMID: 37146214

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

Recent clinical studies

Etiology

Patient-Derived Organoid Models of Human Neuroendocrine Carcinoma.
Dijkstra KK, van den Berg JG, Weeber F, van de Haar J, Velds A, Kaing S, Peters DDGC, Eskens FALM, de Groot DA, Tesselaar MET, Voest EE
Front Endocrinol (Lausanne) 2021;12:627819. Epub 2021 Mar 11 doi: 10.3389/fendo.2021.627819. PMID: 33776923Free PMC Article
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
Molecular subtyping in pancreatic neuroendocrine neoplasms: New insights into clinical, pathological unmet needs and challenges.
Yan J, Yu S, Jia C, Li M, Chen J
Biochim Biophys Acta Rev Cancer 2020 Aug;1874(1):188367. Epub 2020 Apr 25 doi: 10.1016/j.bbcan.2020.188367. PMID: 32339609
Gastroenteropancreatic high-grade neuroendocrine carcinoma.
Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC
Cancer 2014 Sep 15;120(18):2814-23. Epub 2014 Apr 25 doi: 10.1002/cncr.28721. PMID: 24771552
Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma.
Yao JC
Best Pract Res Clin Endocrinol Metab 2007 Mar;21(1):163-72. doi: 10.1016/j.beem.2007.01.006. PMID: 17382271

Diagnosis

Druggable growth dependencies and tumor evolution analysis in patient-derived organoids of neuroendocrine neoplasms from multiple body sites.
Dayton TL, Alcala N, Moonen L, den Hartigh L, Geurts V, Mangiante L, Lap L, Dost AFM, Beumer J, Levy S, van Leeuwaarde RS, Hackeng WM, Samsom K, Voegele C, Sexton-Oates A, Begthel H, Korving J, Hillen L, Brosens LAA, Lantuejoul S, Jaksani S, Kok NFM, Hartemink KJ, Klomp HM, Borel Rinkes IHM, Dingemans AM, Valk GD, Vriens MR, Buikhuisen W, van den Berg J, Tesselaar M, Derks J, Speel EJ, Foll M, Fernández-Cuesta L, Clevers H
Cancer Cell 2023 Dec 11;41(12):2083-2099.e9. doi: 10.1016/j.ccell.2023.11.007. PMID: 38086335
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
Von Hippel-Lindau disease.
Chittiboina P, Lonser RR
Handb Clin Neurol 2015;132:139-56. doi: 10.1016/B978-0-444-62702-5.00010-X. PMID: 26564077Free PMC Article
Gastroenteropancreatic high-grade neuroendocrine carcinoma.
Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC
Cancer 2014 Sep 15;120(18):2814-23. Epub 2014 Apr 25 doi: 10.1002/cncr.28721. PMID: 24771552
von Hippel-Lindau syndrome.
Chou A, Toon C, Pickett J, Gill AJ
Front Horm Res 2013;41:30-49. Epub 2013 Mar 19 doi: 10.1159/000345668. PMID: 23652669

Therapy

Patient-Derived Organoid Models of Human Neuroendocrine Carcinoma.
Dijkstra KK, van den Berg JG, Weeber F, van de Haar J, Velds A, Kaing S, Peters DDGC, Eskens FALM, de Groot DA, Tesselaar MET, Voest EE
Front Endocrinol (Lausanne) 2021;12:627819. Epub 2021 Mar 11 doi: 10.3389/fendo.2021.627819. PMID: 33776923Free PMC Article
Platinum-Based Treatment for Well- and Poorly Differentiated Pancreatic Neuroendocrine Neoplasms.
Pulvirenti A, Raj N, Cingarlini S, Pea A, Tang LH, Luchini C, Chou JF, Grego E, Marinova I, Capanu M, Landoni L, Scarpa A, Allen PJ, Klimstra DS, Reidy-Lagunes DL
Pancreas 2021 Feb 1;50(2):138-146. doi: 10.1097/MPA.0000000000001740. PMID: 33565789Free 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
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
Management of neuroendocrine carcinomas of the pancreas (WHO G3): A tailored approach between proliferation and morphology.
Crippa S, Partelli S, Belfiori G, Palucci M, Muffatti F, Adamenko O, Cardinali L, Doglioni C, Zamboni G, Falconi M
World J Gastroenterol 2016 Dec 7;22(45):9944-9953. doi: 10.3748/wjg.v22.i45.9944. PMID: 28018101Free PMC Article

Prognosis

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
Molecular subtyping in pancreatic neuroendocrine neoplasms: New insights into clinical, pathological unmet needs and challenges.
Yan J, Yu S, Jia C, Li M, Chen J
Biochim Biophys Acta Rev Cancer 2020 Aug;1874(1):188367. Epub 2020 Apr 25 doi: 10.1016/j.bbcan.2020.188367. PMID: 32339609
The Problem of High-Grade Gastroenteropancreatic Neuroendocrine Neoplasms: Well-Differentiated Neuroendocrine Tumors, Neuroendocrine Carcinomas, and Beyond.
Sorbye H, Baudin E, Perren A
Endocrinol Metab Clin North Am 2018 Sep;47(3):683-698. Epub 2018 Jul 11 doi: 10.1016/j.ecl.2018.05.001. PMID: 30098724
Classification of lung neuroendocrine tumors: lights and shadows.
Volante M, Gatti G, Papotti M
Endocrine 2015 Nov;50(2):315-9. Epub 2015 Mar 21 doi: 10.1007/s12020-015-0578-x. PMID: 25795289
Gastroenteropancreatic high-grade neuroendocrine carcinoma.
Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC
Cancer 2014 Sep 15;120(18):2814-23. Epub 2014 Apr 25 doi: 10.1002/cncr.28721. PMID: 24771552

Clinical prediction guides

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
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
Molecular subtyping in pancreatic neuroendocrine neoplasms: New insights into clinical, pathological unmet needs and challenges.
Yan J, Yu S, Jia C, Li M, Chen J
Biochim Biophys Acta Rev Cancer 2020 Aug;1874(1):188367. Epub 2020 Apr 25 doi: 10.1016/j.bbcan.2020.188367. PMID: 32339609
Gastroenteropancreatic high-grade neuroendocrine carcinoma.
Sorbye H, Strosberg J, Baudin E, Klimstra DS, Yao JC
Cancer 2014 Sep 15;120(18):2814-23. Epub 2014 Apr 25 doi: 10.1002/cncr.28721. PMID: 24771552
Neuroendocrine tumors. Molecular targeted therapy for carcinoid and islet-cell carcinoma.
Yao JC
Best Pract Res Clin Endocrinol Metab 2007 Mar;21(1):163-72. doi: 10.1016/j.beem.2007.01.006. PMID: 17382271

Recent systematic reviews

Unraveling molecular pathways of poorly differentiated neuroendocrine carcinomas of the gastroenteropancreatic system: A systematic review.
Girardi DM, Silva ACB, Rêgo JFM, Coudry RA, Riechelmann RP
Cancer Treat Rev 2017 May;56:28-35. Epub 2017 Apr 17 doi: 10.1016/j.ctrv.2017.04.002. PMID: 28456055
Neuroendrocrine tumors of the uterine cervix: A therapeutic challenge for gynecologic oncologists.
Gadducci A, Carinelli S, Aletti G
Gynecol Oncol 2017 Mar;144(3):637-646. Epub 2017 Jan 3 doi: 10.1016/j.ygyno.2016.12.003. PMID: 28057354

Supplemental Content

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    Clinical resources

    Practice guidelines

    • PubMed
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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

    Consumer resources

    Reviews

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