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Liver Large Cell Neuroendocrine Carcinoma

MedGen UID:
1720998
•Concept ID:
C2111730
•
Neoplastic Process
Synonym: Hepatic Large Cell Neuroendocrine Carcinoma

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLiver 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
Clinicopathological, treatment, and prognosis study of 43 gastric neuroendocrine carcinomas.
Liu DJ, Fu XL, Liu W, Zheng LY, Zhang JF, Huo YM, Li J, Hua R, Liu Q, Sun YW
World J Gastroenterol 2017 Jan 21;23(3):516-524. doi: 10.3748/wjg.v23.i3.516. PMID: 28210088Free 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

Pulmonary large cell neuroendocrine carcinoma (LCNEC): a population-based study addressing recent molecular-genetic advances and emerging therapeutic approaches.
Khan J, Yasinzai AQK, Matosz S, Khan M, Heneidi S, Mesa H, Chauhan A, Del Rivero J, Karim NA, Ullah A
Clin Exp Med 2023 Nov;23(7):3947-3955. Epub 2023 Apr 15 doi: 10.1007/s10238-023-01071-8. PMID: 37060529
Risk factors and prognostic factors for pulmonary large cell neuroendocrine carcinoma with brain metastasis.
Chen X, Huang Y, Chen F, She H, Chen X
Cancer Med 2023 Feb;12(4):4087-4099. Epub 2022 Sep 20 doi: 10.1002/cam4.5267. PMID: 36125491Free PMC Article
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
Rovalpituzumab tesirine, a DLL3-targeted antibody-drug conjugate, in recurrent small-cell lung cancer: a first-in-human, first-in-class, open-label, phase 1 study.
Rudin CM, Pietanza MC, Bauer TM, Ready N, Morgensztern D, Glisson BS, Byers LA, Johnson ML, Burris HA 3rd, Robert F, Han TH, Bheddah S, Theiss N, Watson S, Mathur D, Vennapusa B, Zayed H, Lally S, Strickland DK, Govindan R, Dylla SJ, Peng SL, Spigel DR; SCRX16-001 investigators
Lancet Oncol 2017 Jan;18(1):42-51. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30565-4. PMID: 27932068Free PMC Article
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

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
An Algorithmic Immunohistochemical Approach to Define Tumor Type and Assign Site of Origin.
Bellizzi AM
Adv Anat Pathol 2020 May;27(3):114-163. doi: 10.1097/PAP.0000000000000256. PMID: 32205473Free PMC Article
Biliary tract large cell neuroendocrine carcinoma: current evidence.
Raiker R, Chauhan A, Hasanein H, Burkeen G, Horn M, Veedu J, Vela C, Arnold S, Kolesar J, Anthony L, Evers BM, Cavnar M
Orphanet J Rare Dis 2019 Nov 21;14(1):266. doi: 10.1186/s13023-019-1230-2. PMID: 31752927Free PMC Article
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
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

Role of Immunotherapy in Stage IV Large Cell Neuroendocrine Carcinoma of the Lung.
Komiya T, Ravindra N, Powell E
Asian Pac J Cancer Prev 2021 Feb 1;22(2):365-370. doi: 10.31557/APJCP.2021.22.2.365. PMID: 33639649Free PMC Article
Rovalpituzumab tesirine, a DLL3-targeted antibody-drug conjugate, in recurrent small-cell lung cancer: a first-in-human, first-in-class, open-label, phase 1 study.
Rudin CM, Pietanza MC, Bauer TM, Ready N, Morgensztern D, Glisson BS, Byers LA, Johnson ML, Burris HA 3rd, Robert F, Han TH, Bheddah S, Theiss N, Watson S, Mathur D, Vennapusa B, Zayed H, Lally S, Strickland DK, Govindan R, Dylla SJ, Peng SL, Spigel DR; SCRX16-001 investigators
Lancet Oncol 2017 Jan;18(1):42-51. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30565-4. PMID: 27932068Free 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
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
MIBG for diagnosis and therapy of medullary thyroid carcinoma: is there still a role?
Castellani MR, Seregni E, Maccauro M, Chiesa C, Aliberti G, Orunesu E, Bombardieri E
Q J Nucl Med Mol Imaging 2008 Dec;52(4):430-40. PMID: 19088696

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
Biliary tract large cell neuroendocrine carcinoma: current evidence.
Raiker R, Chauhan A, Hasanein H, Burkeen G, Horn M, Veedu J, Vela C, Arnold S, Kolesar J, Anthony L, Evers BM, Cavnar M
Orphanet J Rare Dis 2019 Nov 21;14(1):266. doi: 10.1186/s13023-019-1230-2. PMID: 31752927Free PMC Article
Rovalpituzumab tesirine, a DLL3-targeted antibody-drug conjugate, in recurrent small-cell lung cancer: a first-in-human, first-in-class, open-label, phase 1 study.
Rudin CM, Pietanza MC, Bauer TM, Ready N, Morgensztern D, Glisson BS, Byers LA, Johnson ML, Burris HA 3rd, Robert F, Han TH, Bheddah S, Theiss N, Watson S, Mathur D, Vennapusa B, Zayed H, Lally S, Strickland DK, Govindan R, Dylla SJ, Peng SL, Spigel DR; SCRX16-001 investigators
Lancet Oncol 2017 Jan;18(1):42-51. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30565-4. PMID: 27932068Free 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
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

Pulmonary large cell neuroendocrine carcinoma (LCNEC): a population-based study addressing recent molecular-genetic advances and emerging therapeutic approaches.
Khan J, Yasinzai AQK, Matosz S, Khan M, Heneidi S, Mesa H, Chauhan A, Del Rivero J, Karim NA, Ullah A
Clin Exp Med 2023 Nov;23(7):3947-3955. Epub 2023 Apr 15 doi: 10.1007/s10238-023-01071-8. PMID: 37060529
Risk factors and prognostic factors for pulmonary large cell neuroendocrine carcinoma with brain metastasis.
Chen X, Huang Y, Chen F, She H, Chen X
Cancer Med 2023 Feb;12(4):4087-4099. Epub 2022 Sep 20 doi: 10.1002/cam4.5267. PMID: 36125491Free PMC Article
Clinical characteristics, prognostic factors, and survival trends in esophageal neuroendocrine carcinomas: A population-based study.
Chen C, Hu H, Zheng Z, Yang Y, Chen W, Qiao X, Li P, Zhang S
Cancer Med 2022 Dec;11(24):4935-4945. Epub 2022 May 21 doi: 10.1002/cam4.4829. PMID: 35596661Free PMC Article
Neuroendocrine carcinoma and mixed neuroendocrine‒non-neuroendocrine neoplasm of the stomach: a clinicopathological and exome sequencing study.
Ishida S, Akita M, Fujikura K, Komatsu M, Sawada R, Matsumoto H, Saegusa J, Itoh T, Kakeji Y, Zen Y
Hum Pathol 2021 Apr;110:1-10. Epub 2021 Jan 21 doi: 10.1016/j.humpath.2020.12.008. PMID: 33359239
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

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