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Appendix Large Cell Neuroendocrine Carcinoma

MedGen UID:
473536
•Concept ID:
C2111708
•
Neoplastic Process

Definition

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

Term Hierarchy

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

Recent clinical studies

Etiology

Site-specific trends in gastroenteropancreatic neuroendocrine neoplasms in Bavaria, Germany.
Grundmann N, Voigtländer S, Hakimhashemi A, Pape UF, Meyer M, Müller-Nordhorn J
Cancer Med 2023 Oct;12(19):19949-19958. Epub 2023 Sep 22 doi: 10.1002/cam4.6510. PMID: 37737059Free PMC Article
Clusterin in Neuroendocrine Epithelial Neoplasms: Absence of Expression in a Well-differentiated Tumor Suggests a Jejunoileal Origin.
Czeczok TW, Stashek KM, Maxwell JE, O'Dorisio TM, Howe JR, Hornick JL, Bellizzi AM
Appl Immunohistochem Mol Morphol 2018 Feb;26(2):94-100. doi: 10.1097/PAI.0000000000000563. PMID: 29420353Free PMC Article
Are goblet cell carcinoids a group of heterogeneous tumors?
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
Neuroendocrine tumors involving the gastroenteropancreatic tract: a clinicopathological evaluation of 773 cases.
Estrozi B, Bacchi CE
Clinics (Sao Paulo) 2011;66(10):1671-5. doi: 10.1590/s1807-59322011001000002. PMID: 22012036Free PMC Article
Neuroendocrine carcinomas of the colon and rectum.
Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, Thaler H, Guillem J, Paty P, Cohen AM, Wong WD
Dis Colon Rectum 2004 Feb;47(2):163-9. doi: 10.1007/s10350-003-0038-1. PMID: 15043285

Diagnosis

Site-specific trends in gastroenteropancreatic neuroendocrine neoplasms in Bavaria, Germany.
Grundmann N, Voigtländer S, Hakimhashemi A, Pape UF, Meyer M, Müller-Nordhorn J
Cancer Med 2023 Oct;12(19):19949-19958. Epub 2023 Sep 22 doi: 10.1002/cam4.6510. PMID: 37737059Free PMC Article
Clusterin in Neuroendocrine Epithelial Neoplasms: Absence of Expression in a Well-differentiated Tumor Suggests a Jejunoileal Origin.
Czeczok TW, Stashek KM, Maxwell JE, O'Dorisio TM, Howe JR, Hornick JL, Bellizzi AM
Appl Immunohistochem Mol Morphol 2018 Feb;26(2):94-100. doi: 10.1097/PAI.0000000000000563. PMID: 29420353Free PMC Article
Are goblet cell carcinoids a group of heterogeneous tumors?
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
Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review.
Acosta AM, Wiley EL
Arch Pathol Lab Med 2016 Oct;140(10):1157-62. doi: 10.5858/arpa.2015-0102-RS. PMID: 27684986
Neuroendocrine carcinomas of the colon and rectum.
Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, Thaler H, Guillem J, Paty P, Cohen AM, Wong WD
Dis Colon Rectum 2004 Feb;47(2):163-9. doi: 10.1007/s10350-003-0038-1. PMID: 15043285

Prognosis

Neuroendocrine tumor G3 of bronchopulmonary origin and its classification.
Kasajima A, Klöppel G
Pathol Int 2022 Oct;72(10):488-495. Epub 2022 Aug 19 doi: 10.1111/pin.13266. PMID: 35983917
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Primary Biliary Mixed Adenoneuroendocrine Carcinoma (MANEC): A Short Review.
Acosta AM, Wiley EL
Arch Pathol Lab Med 2016 Oct;140(10):1157-62. doi: 10.5858/arpa.2015-0102-RS. PMID: 27684986
P53, KI-67, CD117 expression in gastrointestinal and pancreatic neuroendocrine tumours and evaluation of their correlation with clinicopathological and prognostic parameters.
Kımıloğlu Şahan E, Erdoğan N, Ulusoy İ, Samet E, Akyıldız İğdem A, Gönüllü D
Turk J Gastroenterol 2015 Mar;26(2):104-11. doi: 10.5152/tjg.2015.1965. PMID: 25835106
Neuroendocrine carcinomas of the colon and rectum.
Bernick PE, Klimstra DS, Shia J, Minsky B, Saltz L, Shi W, Thaler H, Guillem J, Paty P, Cohen AM, Wong WD
Dis Colon Rectum 2004 Feb;47(2):163-9. doi: 10.1007/s10350-003-0038-1. PMID: 15043285

Clinical prediction guides

Pitx2 is a useful marker of midgut-derived neuroendocrine tumours - an immunohistochemical study of 224 cases.
Soukup J, Manethova M, Faistova H, Krbal L, Vitovcova B, Hornychova H, Drugda J, Cesak T, Netuka D, Gabalec F, Ryska A
Histopathology 2022 Dec;81(6):799-807. Epub 2022 Sep 22 doi: 10.1111/his.14789. PMID: 36089904
Appendix Tumor Microenvironment.
Roncati L, Gasparri P, Gallo G, Bernardelli G, Zanelli G, Manenti A
Adv Exp Med Biol 2020;1226:87-95. doi: 10.1007/978-3-030-36214-0_7. PMID: 32030678
Clusterin in Neuroendocrine Epithelial Neoplasms: Absence of Expression in a Well-differentiated Tumor Suggests a Jejunoileal Origin.
Czeczok TW, Stashek KM, Maxwell JE, O'Dorisio TM, Howe JR, Hornick JL, Bellizzi AM
Appl Immunohistochem Mol Morphol 2018 Feb;26(2):94-100. doi: 10.1097/PAI.0000000000000563. PMID: 29420353Free PMC Article
Are goblet cell carcinoids a group of heterogeneous tumors?
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
Novel immunohistochemical localization of 28,000 molecular-weight (Mr) calcium binding protein (calbindin-D28k) in enterochromaffin cells of the human appendix and neuroendocrine tumors (carcinoids and small-cell carcinomas) of the midgut and foregut.
Katsetos CD, Jami MM, Krishna L, Jackson R, Patchefsky AS, Cooper HS
Arch Pathol Lab Med 1994 Jun;118(6):633-9. PMID: 8204010

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