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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 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
The molecular pathogenesis and management of bronchial carcinoids.
Cakir M, Grossman A
Expert Opin Ther Targets 2011 Apr;15(4):457-91. Epub 2011 Jan 29 doi: 10.1517/14728222.2011.555403. PMID: 21275849

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
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 neuroendocrine neoplasms: genes, therapies and models.
Kawasaki K, Fujii M, Sato T
Dis Model Mech 2018 Feb 26;11(2) doi: 10.1242/dmm.029595. PMID: 29590641Free 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

Diagnosis

Adult pancreatoblastoma: Current concepts in pathology.
Omiyale AO
World J Gastroenterol 2021 Jul 14;27(26):4172-4181. doi: 10.3748/wjg.v27.i26.4172. PMID: 34326617Free 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
Pathologic Considerations in Gastroenteropancreatic Neuroendocrine Tumors.
Bellizzi AM
Surg Oncol Clin N Am 2020 Apr;29(2):185-208. doi: 10.1016/j.soc.2019.11.003. PMID: 32151355Free 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
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

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
Peptide Receptor Radionuclide Therapy for Patients With Advanced Lung Carcinoids.
Naraev BG, Ramirez RA, Kendi AT, Halfdanarson TR
Clin Lung Cancer 2019 May;20(3):e376-e392. Epub 2019 Mar 1 doi: 10.1016/j.cllc.2019.02.007. PMID: 30910575
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
Neuroendocrine neoplasms of the gut and pancreas: new insights.
Rindi G, Wiedenmann B
Nat Rev Endocrinol 2011 Aug 2;8(1):54-64. doi: 10.1038/nrendo.2011.120. PMID: 21808296

Prognosis

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
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
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
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
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

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
Neoplasms of the Neuroendocrine Pancreas: An Update in the Classification, Definition, and Molecular Genetic Advances.
Guilmette JM, Nosé V
Adv Anat Pathol 2019 Jan;26(1):13-30. doi: 10.1097/PAP.0000000000000201. PMID: 29912000
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

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

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