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Appendix Small Cell Neuroendocrine Carcinoma

MedGen UID:
310473
Concept ID:
C1706833
Neoplastic Process
Synonym: Appendix Small Cell Carcinoma

Definition

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

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAppendix Small Cell Neuroendocrine Carcinoma

Professional guidelines

PubMed

Daly ME, Singh N, Ismaila N; Management of Stage III NSCLC Guideline Expert Panel
J Clin Oncol 2024 Sep 1;42(25):3058-3060. Epub 2024 Jul 23 doi: 10.1200/JCO-24-01324. PMID: 39042842
Singh N, Daly ME, Ismaila N; Management of Stage III NSCLC Guideline Expert Panel
J Clin Oncol 2023 Sep 20;41(27):4430-4432. Epub 2023 Jul 20 doi: 10.1200/JCO.23.01261. PMID: 37471673
Owen DH, Singh N, Ismaila N, Masters G, Riely GJ, Robinson AG, Schneider BJ, Jaiyesimi IA
J Clin Oncol 2023 Aug 20;41(24):e63-e72. Epub 2023 Jul 11 doi: 10.1200/JCO.23.01055. PMID: 37433095

Recent clinical studies

Etiology

Okumura T, Noguchi T, Sekiguchi N, Kobayashi T, Kanda S, Ida K, Minagawa T, Tokumaru S, Umemura T, Koizumi T
Chemotherapy 2023;68(2):87-94. Epub 2022 Nov 3 doi: 10.1159/000527377. PMID: 36327945
Hellmann MD, Rizvi NA, Goldman JW, Gettinger SN, Borghaei H, Brahmer JR, Ready NE, Gerber DE, Chow LQ, Juergens RA, Shepherd FA, Laurie SA, Geese WJ, Agrawal S, Young TC, Li X, Antonia SJ
Lancet Oncol 2017 Jan;18(1):31-41. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30624-6. PMID: 27932067Free PMC Article
Capozzi M, Caterina I, De Divitiis C, von Arx C, Maiolino P, Tatangelo F, Cavalcanti E, Di Girolamo E, Iaffaioli RV, Scala S, Tafuto S; ENETS Center of Excellence Multidisciplinary Group for Neuroendocrine Tumors in Naples (Italy)
Int J Surg 2015 Sep;21 Suppl 1:S89-94. Epub 2015 Jun 27 doi: 10.1016/j.ijsu.2015.06.064. PMID: 26123382
Garon EB, Rizvi NA, Hui R, Leighl N, Balmanoukian AS, Eder JP, Patnaik A, Aggarwal C, Gubens M, Horn L, Carcereny E, Ahn MJ, Felip E, Lee JS, Hellmann MD, Hamid O, Goldman JW, Soria JC, Dolled-Filhart M, Rutledge RZ, Zhang J, Lunceford JK, Rangwala R, Lubiniecki GM, Roach C, Emancipator K, Gandhi L; KEYNOTE-001 Investigators
N Engl J Med 2015 May 21;372(21):2018-28. Epub 2015 Apr 19 doi: 10.1056/NEJMoa1501824. PMID: 25891174
Carr NJ, Sobin LH
Semin Diagn Pathol 2004 May;21(2):108-19. doi: 10.1053/j.semdp.2004.11.004. PMID: 15807471

Diagnosis

Okumura T, Noguchi T, Sekiguchi N, Kobayashi T, Kanda S, Ida K, Minagawa T, Tokumaru S, Umemura T, Koizumi T
Chemotherapy 2023;68(2):87-94. Epub 2022 Nov 3 doi: 10.1159/000527377. PMID: 36327945
Wang Q, Zhou T, Sun H, Li B
Cancer Biol Ther 2017 Sep 2;18(9):666-669. doi: 10.1080/15384047.2017.1360447. PMID: 28886266Free PMC Article
Acosta AM, Wiley EL
Arch Pathol Lab Med 2016 Oct;140(10):1157-62. doi: 10.5858/arpa.2015-0102-RS. PMID: 27684986
Capozzi M, Caterina I, De Divitiis C, von Arx C, Maiolino P, Tatangelo F, Cavalcanti E, Di Girolamo E, Iaffaioli RV, Scala S, Tafuto S; ENETS Center of Excellence Multidisciplinary Group for Neuroendocrine Tumors in Naples (Italy)
Int J Surg 2015 Sep;21 Suppl 1:S89-94. Epub 2015 Jun 27 doi: 10.1016/j.ijsu.2015.06.064. PMID: 26123382
Garon EB, Rizvi NA, Hui R, Leighl N, Balmanoukian AS, Eder JP, Patnaik A, Aggarwal C, Gubens M, Horn L, Carcereny E, Ahn MJ, Felip E, Lee JS, Hellmann MD, Hamid O, Goldman JW, Soria JC, Dolled-Filhart M, Rutledge RZ, Zhang J, Lunceford JK, Rangwala R, Lubiniecki GM, Roach C, Emancipator K, Gandhi L; KEYNOTE-001 Investigators
N Engl J Med 2015 May 21;372(21):2018-28. Epub 2015 Apr 19 doi: 10.1056/NEJMoa1501824. PMID: 25891174

Therapy

Liu CY, Lin HF, Lai WY, Lin YY, Lin TW, Yang YP, Tsai FT, Wang CL, Luo YH, Chen YM, Hsu PK, Kai LJ, Kiat AOH, Chien Y, Chiou SH, Wang CY
J Chin Med Assoc 2022 Apr 1;85(4):409-413. doi: 10.1097/JCMA.0000000000000703. PMID: 35383703
ESMO Guidelines Committee
Ann Oncol 2017 Jul 1;28(suppl_4):iv158-iv161. doi: 10.1093/annonc/mdx245. PMID: 28881928
Hellmann MD, Rizvi NA, Goldman JW, Gettinger SN, Borghaei H, Brahmer JR, Ready NE, Gerber DE, Chow LQ, Juergens RA, Shepherd FA, Laurie SA, Geese WJ, Agrawal S, Young TC, Li X, Antonia SJ
Lancet Oncol 2017 Jan;18(1):31-41. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30624-6. PMID: 27932067Free PMC Article
Capozzi M, Caterina I, De Divitiis C, von Arx C, Maiolino P, Tatangelo F, Cavalcanti E, Di Girolamo E, Iaffaioli RV, Scala S, Tafuto S; ENETS Center of Excellence Multidisciplinary Group for Neuroendocrine Tumors in Naples (Italy)
Int J Surg 2015 Sep;21 Suppl 1:S89-94. Epub 2015 Jun 27 doi: 10.1016/j.ijsu.2015.06.064. PMID: 26123382
Garon EB, Rizvi NA, Hui R, Leighl N, Balmanoukian AS, Eder JP, Patnaik A, Aggarwal C, Gubens M, Horn L, Carcereny E, Ahn MJ, Felip E, Lee JS, Hellmann MD, Hamid O, Goldman JW, Soria JC, Dolled-Filhart M, Rutledge RZ, Zhang J, Lunceford JK, Rangwala R, Lubiniecki GM, Roach C, Emancipator K, Gandhi L; KEYNOTE-001 Investigators
N Engl J Med 2015 May 21;372(21):2018-28. Epub 2015 Apr 19 doi: 10.1056/NEJMoa1501824. PMID: 25891174

Prognosis

Okumura T, Noguchi T, Sekiguchi N, Kobayashi T, Kanda S, Ida K, Minagawa T, Tokumaru S, Umemura T, Koizumi T
Chemotherapy 2023;68(2):87-94. Epub 2022 Nov 3 doi: 10.1159/000527377. PMID: 36327945
Zhang K, Meyerson C, Kassardjian A, Westbrook LM, Zheng W, Wang HL
Adv Anat Pathol 2019 Mar;26(2):75-83. doi: 10.1097/PAP.0000000000000222. PMID: 30601149
Hellmann MD, Rizvi NA, Goldman JW, Gettinger SN, Borghaei H, Brahmer JR, Ready NE, Gerber DE, Chow LQ, Juergens RA, Shepherd FA, Laurie SA, Geese WJ, Agrawal S, Young TC, Li X, Antonia SJ
Lancet Oncol 2017 Jan;18(1):31-41. Epub 2016 Dec 5 doi: 10.1016/S1470-2045(16)30624-6. PMID: 27932067Free PMC Article
Acosta AM, Wiley EL
Arch Pathol Lab Med 2016 Oct;140(10):1157-62. doi: 10.5858/arpa.2015-0102-RS. PMID: 27684986
Rindi G, Inzani F, Solcia E
Endocrinol Metab Clin North Am 2010 Dec;39(4):713-27. doi: 10.1016/j.ecl.2010.08.009. PMID: 21095540

Clinical prediction guides

Zehani A, Aloui S, Chelly I, Haouet S, Kchir N
Tunis Med 2017 Jun;95(6):393-400. PMID: 29512793
Macak J, Nemejcova K, Dvorackova J
Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub 2017 Sep;161(3):281-285. Epub 2017 Jun 12 doi: 10.5507/bp.2017.027. PMID: 28659644
Ayub A, Parkash O, Santana-Rodríguez N, Raad W, Bhora FY
Indian J Gastroenterol 2016 Sep;35(5):354-360. Epub 2016 Sep 6 doi: 10.1007/s12664-016-0687-3. PMID: 27595862
Garon EB, Rizvi NA, Hui R, Leighl N, Balmanoukian AS, Eder JP, Patnaik A, Aggarwal C, Gubens M, Horn L, Carcereny E, Ahn MJ, Felip E, Lee JS, Hellmann MD, Hamid O, Goldman JW, Soria JC, Dolled-Filhart M, Rutledge RZ, Zhang J, Lunceford JK, Rangwala R, Lubiniecki GM, Roach C, Emancipator K, Gandhi L; KEYNOTE-001 Investigators
N Engl J Med 2015 May 21;372(21):2018-28. Epub 2015 Apr 19 doi: 10.1056/NEJMoa1501824. PMID: 25891174
Rindi G, Inzani F, Solcia E
Endocrinol Metab Clin North Am 2010 Dec;39(4):713-27. doi: 10.1016/j.ecl.2010.08.009. PMID: 21095540

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