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Pancreatic small cell neuroendocrine carcinoma

MedGen UID:
459466
Concept ID:
C2987240
Neoplastic Process
Synonyms: endocrine pancreas small cell carcinoma; Pancreatic Small Cell Carcinoma; Pancreatic Small Cell NEC; pancreatic small cell NEC; Pancreatic Small Cell NEC G3; pancreatic small cell NEC G3; Pancreatic Small Cell Neuroendocrine Carcinoma; pancreatic small cell neuroendocrine carcinoma; small cell carcinoma of endocrine pancreas
 
Monarch Initiative: MONDO:0006348

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 small cells. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Harris PE, Zhernosekov K
Front Endocrinol (Lausanne) 2022;13:941832. Epub 2022 Oct 31 doi: 10.3389/fendo.2022.941832. PMID: 36387893Free PMC Article
Buscail L, Bournet B, Cordelier P
Nat Rev Gastroenterol Hepatol 2020 Mar;17(3):153-168. Epub 2020 Jan 31 doi: 10.1038/s41575-019-0245-4. PMID: 32005945
Ciardiello F, Tortora G
N Engl J Med 2008 Mar 13;358(11):1160-74. doi: 10.1056/NEJMra0707704. PMID: 18337605

Curated

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

Recent clinical studies

Etiology

Nakamura K, Zhu Z, Roy S, Jun E, Han H, Munoz RM, Nishiwada S, Sharma G, Cridebring D, Zenhausern F, Kim S, Roe DJ, Darabi S, Han IW, Evans DB, Yamada S, Demeure MJ, Becerra C, Celinski SA, Borazanci E, Tsai S, Kodera Y, Park JO, Bolton JS, Wang X, Kim SC, Von Hoff D, Goel A
Gastroenterology 2022 Nov;163(5):1252-1266.e2. Epub 2022 Jul 16 doi: 10.1053/j.gastro.2022.06.090. PMID: 35850192Free PMC Article
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
Rüschoff J, Nagelmeier I, Jasani B, Stoss O
Pathologe 2021 Nov;42(Suppl 1):62-68. Epub 2020 Dec 21 doi: 10.1007/s00292-020-00878-6. PMID: 33346874
Jonna S, Feldman RA, Swensen J, Gatalica Z, Korn WM, Borghaei H, Ma PC, Nieva JJ, Spira AI, Vanderwalde AM, Wozniak AJ, Kim ES, Liu SV
Clin Cancer Res 2019 Aug 15;25(16):4966-4972. Epub 2019 Apr 15 doi: 10.1158/1078-0432.CCR-19-0160. PMID: 30988082Free PMC Article
Aggarwal R, Huang J, Alumkal JJ, Zhang L, Feng FY, Thomas GV, Weinstein AS, Friedl V, Zhang C, Witte ON, Lloyd P, Gleave M, Evans CP, Youngren J, Beer TM, Rettig M, Wong CK, True L, Foye A, Playdle D, Ryan CJ, Lara P, Chi KN, Uzunangelov V, Sokolov A, Newton Y, Beltran H, Demichelis F, Rubin MA, Stuart JM, Small EJ
J Clin Oncol 2018 Aug 20;36(24):2492-2503. Epub 2018 Jul 9 doi: 10.1200/JCO.2017.77.6880. PMID: 29985747Free PMC Article

Diagnosis

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
Park SY, Choi KH, Chon HK
Korean J Intern Med 2020 Mar;35(2):478-479. Epub 2018 Oct 16 doi: 10.3904/kjim.2018.262. PMID: 30317845Free PMC Article
El Rassy E, Tabchi S, Kourie HR, Assi T, Chebib R, Farhat F, Kattan J
Clin Res Hepatol Gastroenterol 2016 Jun;40(3):276-280. Epub 2015 Nov 11 doi: 10.1016/j.clinre.2015.09.010. PMID: 26566245
Chan JK
Int J Surg Pathol 2014 Feb;22(1):12-32. Epub 2014 Jan 9 doi: 10.1177/1066896913517939. PMID: 24406626
Pepe G, Moncayo R, Bombardieri E, Chiti A
Eur J Nucl Med Mol Imaging 2012 Feb;39 Suppl 1:S41-51. doi: 10.1007/s00259-011-2019-2. PMID: 22388628

Therapy

Singhal A, Li BT, O'Reilly EM
Nat Med 2024 Apr;30(4):969-983. Epub 2024 Apr 18 doi: 10.1038/s41591-024-02903-0. PMID: 38637634
Wei D, Wang L, Zuo X, Maitra A, Bresalier RS
Clin Cancer Res 2024 Feb 16;30(4):655-662. doi: 10.1158/1078-0432.CCR-23-2098. PMID: 37831007Free PMC Article
Pandit B, Royzen M
Genes (Basel) 2022 Mar 5;13(3) doi: 10.3390/genes13030466. PMID: 35328020Free PMC Article
Gao H, Korn JM, Ferretti S, Monahan JE, Wang Y, Singh M, Zhang C, Schnell C, Yang G, Zhang Y, Balbin OA, Barbe S, Cai H, Casey F, Chatterjee S, Chiang DY, Chuai S, Cogan SM, Collins SD, Dammassa E, Ebel N, Embry M, Green J, Kauffmann A, Kowal C, Leary RJ, Lehar J, Liang Y, Loo A, Lorenzana E, Robert McDonald E 3rd, McLaughlin ME, Merkin J, Meyer R, Naylor TL, Patawaran M, Reddy A, Röelli C, Ruddy DA, Salangsang F, Santacroce F, Singh AP, Tang Y, Tinetto W, Tobler S, Velazquez R, Venkatesan K, Von Arx F, Wang HQ, Wang Z, Wiesmann M, Wyss D, Xu F, Bitter H, Atadja P, Lees E, Hofmann F, Li E, Keen N, Cozens R, Jensen MR, Pryer NK, Williams JA, Sellers WR
Nat Med 2015 Nov;21(11):1318-25. Epub 2015 Oct 19 doi: 10.1038/nm.3954. PMID: 26479923
Ciardiello F, Tortora G
N Engl J Med 2008 Mar 13;358(11):1160-74. doi: 10.1056/NEJMra0707704. PMID: 18337605

Prognosis

Quintanal-Villalonga A, Taniguchi H, Zhan YA, Hasan MM, Chavan SS, Meng F, Uddin F, Manoj P, Donoghue MTA, Won HH, Chan JM, Ciampricotti M, Chow A, Offin M, Chang JC, Ray-Kirton J, Tischfield SE, Egger J, Bhanot UK, Linkov I, Asher M, Sinha S, Silber J, Iacobuzio-Donahue CA, Roehrl MH, Hollmann TJ, Yu HA, Qiu J, de Stanchina E, Baine MK, Rekhtman N, Poirier JT, Loomis B, Koche RP, Rudin CM, Sen T
Cancer Discov 2021 Dec 1;11(12):3028-3047. doi: 10.1158/2159-8290.CD-20-1863. PMID: 34155000Free PMC Article
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
Elosua-Bayes M, Nieto P, Mereu E, Gut I, Heyn H
Nucleic Acids Res 2021 May 21;49(9):e50. doi: 10.1093/nar/gkab043. PMID: 33544846Free PMC Article
Steins M, Thomas M, Geißler M
Recent Results Cancer Res 2018;211:1-17. doi: 10.1007/978-3-319-91442-8_1. PMID: 30069756
Steins M, Thomas M, Geissler M
Recent Results Cancer Res 2014;201:109-23. doi: 10.1007/978-3-642-54490-3_6. PMID: 24756788

Clinical prediction guides

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
Elosua-Bayes M, Nieto P, Mereu E, Gut I, Heyn H
Nucleic Acids Res 2021 May 21;49(9):e50. doi: 10.1093/nar/gkab043. PMID: 33544846Free PMC Article
Steins M, Thomas M, Geißler M
Recent Results Cancer Res 2018;211:1-17. doi: 10.1007/978-3-319-91442-8_1. PMID: 30069756
Gao H, Korn JM, Ferretti S, Monahan JE, Wang Y, Singh M, Zhang C, Schnell C, Yang G, Zhang Y, Balbin OA, Barbe S, Cai H, Casey F, Chatterjee S, Chiang DY, Chuai S, Cogan SM, Collins SD, Dammassa E, Ebel N, Embry M, Green J, Kauffmann A, Kowal C, Leary RJ, Lehar J, Liang Y, Loo A, Lorenzana E, Robert McDonald E 3rd, McLaughlin ME, Merkin J, Meyer R, Naylor TL, Patawaran M, Reddy A, Röelli C, Ruddy DA, Salangsang F, Santacroce F, Singh AP, Tang Y, Tinetto W, Tobler S, Velazquez R, Venkatesan K, Von Arx F, Wang HQ, Wang Z, Wiesmann M, Wyss D, Xu F, Bitter H, Atadja P, Lees E, Hofmann F, Li E, Keen N, Cozens R, Jensen MR, Pryer NK, Williams JA, Sellers WR
Nat Med 2015 Nov;21(11):1318-25. Epub 2015 Oct 19 doi: 10.1038/nm.3954. PMID: 26479923
Chan JK
Int J Surg Pathol 2014 Feb;22(1):12-32. Epub 2014 Jan 9 doi: 10.1177/1066896913517939. PMID: 24406626

Recent systematic reviews

Silva A, Cerqueira MC, Rosa B, Sobral C, Pinto-Ribeiro F, Costa MF, Baltazar F, Afonso J
Int J Mol Sci 2023 Mar 7;24(6) doi: 10.3390/ijms24065141. PMID: 36982217Free PMC Article
Arman T, Nelson PS
Front Endocrinol (Lausanne) 2022;13:1012005. Epub 2022 Nov 11 doi: 10.3389/fendo.2022.1012005. PMID: 36440195Free PMC Article
Shuai Y, Ma Z, Lu J, Feng J
Cell Prolif 2020 Jan;53(1):e12716. Epub 2019 Nov 27 doi: 10.1111/cpr.12716. PMID: 31774607Free PMC Article
Isidori AM, Lenzi A
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1217-25. doi: 10.1590/s0004-27302007000800007. PMID: 18209859
Barlési F, Villani P, Doddoli C, Gimenez C, Kleisbauer JP
Fundam Clin Pharmacol 2004 Feb;18(1):85-91. doi: 10.1046/j.0767-3981.2003.00206.x. PMID: 14748759

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    Clinical resources

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

    Curated

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

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