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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

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
Liver resection for non-colorectal, non-carcinoid, non-sarcoma metastases: a multicenter study.
Martel G, Hawel J, Rekman J, Croome KP, Bertens K, Balaa FK, Hernandez-Alejandro R
PLoS One 2015;10(3):e0120569. Epub 2015 Mar 26 doi: 10.1371/journal.pone.0120569. PMID: 25811923Free 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
Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis.
Tanaka S, Yamasaki S, Matsushita H, Ozawa Y, Kurosaki A, Takeuchi K, Hoshihara Y, Doi T, Watanabe G, Kawaminami K
Pathol Int 2000 Feb;50(2):146-52. doi: 10.1046/j.1440-1827.2000.01016.x. PMID: 10792774

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
Malignant Insulinoma Is Largely Derived From Nonfunctioning Pancreatic Neuroendocrine Tumors: A Contemporary View.
Yu R
Pancreas 2020 Jul;49(6):733-736. doi: 10.1097/MPA.0000000000001562. PMID: 32590616
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
Pancreatic somatostatinoma.
Sakazaki S, Umeyama K, Nakagawa H, Hashimoto H, Kamino K, Mitsuhashi T, Yamaguchi K
Am J Surg 1983 Nov;146(5):674-9. doi: 10.1016/0002-9610(83)90310-0. PMID: 6314831

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
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
An Unusual Case of an Extremely Large α-Fetoprotein-Producing Tumor.
Takahashi K, Tsuchiya A, Terai S
Gastroenterology 2016 Dec;151(6):1077-1080. Epub 2016 Oct 27 doi: 10.1053/j.gastro.2016.08.018. PMID: 28340919
Amelioration of symptoms and reduction of VIP levels after hepatic artery chemoembolization in a patient with sandostatin resistant VIPoma.
Shaib W, Mitchell K, Saif MW
Yale J Biol Med 2010 Mar;83(1):27-33. PMID: 20351979Free PMC Article

Prognosis

All Together Now: Standardization of Nomenclature for Neuroendocrine Neoplasms across Multiple Organs.
Jafari P, Husain AN, Setia N
Surg Pathol Clin 2023 Mar;16(1):131-150. Epub 2022 Dec 9 doi: 10.1016/j.path.2022.09.012. PMID: 36739160
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
Liver resection for non-colorectal, non-carcinoid, non-sarcoma metastases: a multicenter study.
Martel G, Hawel J, Rekman J, Croome KP, Bertens K, Balaa FK, Hernandez-Alejandro R
PLoS One 2015;10(3):e0120569. Epub 2015 Mar 26 doi: 10.1371/journal.pone.0120569. PMID: 25811923Free 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
Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis.
Tanaka S, Yamasaki S, Matsushita H, Ozawa Y, Kurosaki A, Takeuchi K, Hoshihara Y, Doi T, Watanabe G, Kawaminami K
Pathol Int 2000 Feb;50(2):146-52. doi: 10.1046/j.1440-1827.2000.01016.x. PMID: 10792774

Clinical prediction guides

ABCD1 as a Novel Diagnostic Marker for Solid Pseudopapillary Neoplasm of the Pancreas.
Liu YA, Liu Y, Tu J, Shi Y, Pang J, Huang Q, Wang X, Lin Z, Zhao Y, Wang W, Peng J, Wu W
Am J Surg Pathol 2024 May 1;48(5):511-520. Epub 2024 Apr 3 doi: 10.1097/PAS.0000000000002205. PMID: 38567813Free 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
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
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

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

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    Curated

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

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    Reviews

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