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Colorectal Large Cell Neuroendocrine Carcinoma

MedGen UID:
474241
•Concept ID:
C3272608
•
Neoplastic Process
Synonym: Large Intestinal Large Cell Neuroendocrine Carcinoma

Definition

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

Term Hierarchy

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

Professional guidelines

PubMed

Morphology Matters: A Critical Reappraisal of the Clinical Relevance of Morphologic Criteria From the 2019 WHO Classification in a Large Colorectal Cancer Cohort Comprising 1004 Cases.
Jesinghaus M, Schmitt M, Lang C, Reiser M, Scheiter A, Konukiewitz B, Steiger K, Silva M, Tschurtschenthaler M, Lange S, Foersch S, Becker KF, Saur D, Friess H, Halfter K, Engel J, Boxberg M, Pfarr N, Wilhelm D, Weichert W
Am J Surg Pathol 2021 Jul 1;45(7):969-978. doi: 10.1097/PAS.0000000000001692. PMID: 34105518
Value of thyroid transcription factor-1 and surfactant apoprotein A in the differential diagnosis of pulmonary carcinomas: a study of 109 cases.
Zamecnik J, Kodet R
Virchows Arch 2002 Apr;440(4):353-61. Epub 2001 Oct 19 doi: 10.1007/s00428-001-0552-2. PMID: 11956814
Regional treatment of hepatic metastases and hepatocellular carcinoma.
Kemeny N, Schneider A
Curr Probl Cancer 1989 Jul-Aug;13(4):197-283. doi: 10.1016/0147-0272(89)90007-x. PMID: 2548812

Recent clinical studies

Etiology

WNT2 activation through proximal germline deletion predisposes to small intestinal neuroendocrine tumors and intestinal adenocarcinomas.
Aavikko M, Kaasinen E, Andersson N, Pentinmikko N, Sulo P, Donner I, Pihlajamaa P, Kuosmanen A, Bramante S, Katainen R, Sipilä LJ, Martin S, Arola J, Carpén O, Heiskanen I, Mecklin JP, Taipale J, Ristimäki A, Lehti K, Gucciardo E, Katajisto P, Schalin-Jäntti C, Vahteristo P, Aaltonen LA
Hum Mol Genet 2021 Nov 30;30(24):2429-2440. doi: 10.1093/hmg/ddab206. PMID: 34274970Free PMC Article
(18)F-FDG-Avid Thyroid Incidentalomas: The Importance of Contextual Interpretation.
Pattison DA, Bozin M, Gorelik A, Hofman MS, Hicks RJ, Skandarajah A
J Nucl Med 2018 May;59(5):749-755. Epub 2017 Oct 12 doi: 10.2967/jnumed.117.198085. PMID: 29025986
Aggressive large-cell neuroendocrine carcinoma of the sigmoid colon in a patient with ulcerative colitis.
Stoner P, Ghaffaripour T, Cohen D
BMJ Case Rep 2017 Aug 7;2017 doi: 10.1136/bcr-2017-220093. PMID: 28790093Free PMC Article
Poorly-differentiated colorectal neuroendocrine tumour: CT differentiation from well-differentiated neuroendocrine tumour and poorly-differentiated adenocarcinomas.
Kang JH, Kim SH, Han JK
Eur Radiol 2017 Sep;27(9):3867-3876. Epub 2017 Feb 16 doi: 10.1007/s00330-017-4764-5. PMID: 28210802
The epidemiology and pathogenesis of neoplasia in the small intestine.
Schottenfeld D, Beebe-Dimmer JL, Vigneau FD
Ann Epidemiol 2009 Jan;19(1):58-69. doi: 10.1016/j.annepidem.2008.10.004. PMID: 19064190Free PMC Article

Diagnosis

POU2F3: A Sensitive and Specific Diagnostic Marker for Neuroendocrine-low/negative Small Cell Lung Cancer.
Wang Y, Jin Y, Shen X, Zheng Q, Xue Q, Chen L, Lin Y, Li Y
Am J Surg Pathol 2023 Sep 1;47(9):1059-1066. Epub 2023 Jun 26 doi: 10.1097/PAS.0000000000002081. PMID: 37357936
Multiple large cell neuroendocrine carcinomas in the rectum and descending colon presented with liver metastasis: A case report.
Fukushima N, Furukawa K, Takano Y, Suzuki T, Yoshinobu M, Hanyu K, Kobayashi S
Asian J Endosc Surg 2022 Apr;15(2):405-409. Epub 2022 Jan 9 doi: 10.1111/ases.13025. PMID: 35001529
Aggressive large-cell neuroendocrine carcinoma of the sigmoid colon in a patient with ulcerative colitis.
Stoner P, Ghaffaripour T, Cohen D
BMJ Case Rep 2017 Aug 7;2017 doi: 10.1136/bcr-2017-220093. PMID: 28790093Free PMC Article
Poorly-differentiated colorectal neuroendocrine tumour: CT differentiation from well-differentiated neuroendocrine tumour and poorly-differentiated adenocarcinomas.
Kang JH, Kim SH, Han JK
Eur Radiol 2017 Sep;27(9):3867-3876. Epub 2017 Feb 16 doi: 10.1007/s00330-017-4764-5. PMID: 28210802
Updates in the Pathologic Diagnosis and Classification of Epithelial Neoplasms of Urachal Origin.
Paner GP, Lopez-Beltran A, Sirohi D, Amin MB
Adv Anat Pathol 2016 Mar;23(2):71-83. doi: 10.1097/PAP.0000000000000110. PMID: 26849813

Therapy

Colorectal Large-Cell Neuroendocrine Carcinoma: Case Report and Literature Review for Potential Novel Therapeutic Options.
Chen X, Wang X, Abadi M, Kumar A
Clin Colorectal Cancer 2020 Mar;19(1):61-64. Epub 2019 Oct 31 doi: 10.1016/j.clcc.2019.10.005. PMID: 31761668
Survival in Patients with High-Grade Colorectal Neuroendocrine Carcinomas: The Role of Surgery and Chemotherapy.
Fields AC, Lu P, Vierra BM, Hu F, Irani J, Bleday R, Goldberg JE, Nash GM, Melnitchouk N
Ann Surg Oncol 2019 Apr;26(4):1127-1133. Epub 2019 Jan 31 doi: 10.1245/s10434-019-07203-3. PMID: 30706232Free PMC Article
Aggressive large-cell neuroendocrine carcinoma of the sigmoid colon in a patient with ulcerative colitis.
Stoner P, Ghaffaripour T, Cohen D
BMJ Case Rep 2017 Aug 7;2017 doi: 10.1136/bcr-2017-220093. PMID: 28790093Free PMC Article
Heterogeneity of colorectal cancer risk by tumour characteristics: Large prospective study of UK women.
Burón Pust A, Alison R, Blanks R, Pirie K, Gaitskell K, Barnes I, Gathani T, Reeves G, Beral V, Green J; Million Women Study Collaborators
Int J Cancer 2017 Mar 1;140(5):1082-1090. doi: 10.1002/ijc.30527. PMID: 27859268Free PMC Article
Regional treatment of hepatic metastases and hepatocellular carcinoma.
Kemeny N, Schneider A
Curr Probl Cancer 1989 Jul-Aug;13(4):197-283. doi: 10.1016/0147-0272(89)90007-x. PMID: 2548812

Prognosis

Genomic characterization reveals distinct mutation landscapes and therapeutic implications in neuroendocrine carcinomas of the gastrointestinal tract.
Wu H, Yu Z, Liu Y, Guo L, Teng L, Guo L, Liang L, Wang J, Gao J, Li R, Yang L, Nie X, Su D, Liang Z
Cancer Commun (Lond) 2022 Dec;42(12):1367-1386. Epub 2022 Oct 20 doi: 10.1002/cac2.12372. PMID: 36264285Free PMC Article
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
Colorectal large-cell neuroendocrine carcinoma with lymphoid stroma: further evidence confirming a unique subtype associated with MLH1/PMS2 loss, BRAF mutation, Epstein-Barr virus negativity, and the possibility of a better prognosis.
Chetty R, Capo-Chichi JM, Serra S
Histopathology 2019 Aug;75(2):247-253. Epub 2019 Jul 2 doi: 10.1111/his.13875. PMID: 30990915
Poorly-differentiated colorectal neuroendocrine tumour: CT differentiation from well-differentiated neuroendocrine tumour and poorly-differentiated adenocarcinomas.
Kang JH, Kim SH, Han JK
Eur Radiol 2017 Sep;27(9):3867-3876. Epub 2017 Feb 16 doi: 10.1007/s00330-017-4764-5. PMID: 28210802
The epidemiology and pathogenesis of neoplasia in the small intestine.
Schottenfeld D, Beebe-Dimmer JL, Vigneau FD
Ann Epidemiol 2009 Jan;19(1):58-69. doi: 10.1016/j.annepidem.2008.10.004. PMID: 19064190Free PMC Article

Clinical prediction guides

POU2F3: A Sensitive and Specific Diagnostic Marker for Neuroendocrine-low/negative Small Cell Lung Cancer.
Wang Y, Jin Y, Shen X, Zheng Q, Xue Q, Chen L, Lin Y, Li Y
Am J Surg Pathol 2023 Sep 1;47(9):1059-1066. Epub 2023 Jun 26 doi: 10.1097/PAS.0000000000002081. PMID: 37357936
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
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
Updates in the Pathologic Diagnosis and Classification of Epithelial Neoplasms of Urachal Origin.
Paner GP, Lopez-Beltran A, Sirohi D, Amin MB
Adv Anat Pathol 2016 Mar;23(2):71-83. doi: 10.1097/PAP.0000000000000110. PMID: 26849813
Biology of colorectal and gastric cancer cell lines.
Park JG, Gazdar AF
J Cell Biochem Suppl 1996;24:131-41. doi: 10.1002/jcb.240630508. PMID: 8806095

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

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