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Classic neuroendocrine tumor of appendix

MedGen UID:
1843245
Concept ID:
C5679854
Neoplastic Process
Synonyms: Classic appendiceal neuroendocrine tumor; classic appendiceal neuroendocrine tumor; classic appendiceal neuroendocrine tumour; Classic appendix neuroendocrine tumor; classic appendix neuroendocrine tumor; classic appendix neuroendocrine tumour; classic neuroendocrine tumor of appendix
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018016
Orphanet: ORPHA329977

Definition

A rare endocrine tumor of the appendix, seen twice as frequently in females than in males, and usually presenting before the fifth decade of life. It is usually asymptomatic when located in the tip of the appendix (without obstruction), but acute appendicitis is often associated. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClassic neuroendocrine tumor of appendix

Professional guidelines

PubMed

McConnell YJ, Mack LA, Gui X, Carr NJ, Sideris L, Temple WJ, Dubé P, Chandrakumaran K, Moran BJ, Cecil TD
Ann Surg Oncol 2014 Jun;21(6):1975-82. Epub 2014 Jan 8 doi: 10.1245/s10434-013-3469-5. PMID: 24398544
Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC; North American Neuroendocrine Tumor Society (NANETS)
Pancreas 2010 Aug;39(6):753-66. doi: 10.1097/MPA.0b013e3181ebb2a5. PMID: 20664473
O'Donnell ME, Carson J, Garstin WI
Int J Clin Pract 2007 Mar;61(3):431-7. Epub 2006 Aug 15 doi: 10.1111/j.1742-1241.2006.00875.x. PMID: 16911574

Recent clinical studies

Etiology

Mikaeel RR, Young JP, Tapia Rico G, Hewett PJ, Hardingham JE, Uylaki W, Horsnell M, Price TJ
Crit Rev Clin Lab Sci 2021 Sep;58(6):369-384. Epub 2021 Feb 11 doi: 10.1080/10408363.2021.1881756. PMID: 33569997
Şenel F, Karaman H, Demir H
Turk J Med Sci 2018 Feb 23;48(1):68-73. doi: 10.3906/sag-1709-37. PMID: 29479957
Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC; North American Neuroendocrine Tumor Society (NANETS)
Pancreas 2010 Aug;39(6):753-66. doi: 10.1097/MPA.0b013e3181ebb2a5. PMID: 20664473
Deschamps L, Couvelard A
Arch Pathol Lab Med 2010 Jun;134(6):871-5. doi: 10.5858/134.6.871. PMID: 20524865
Nakano PH, Bloom RR, Brown BC, Gray SW, Skandalakis JE, Kibbe JM
Am Surg 1987 Sep;53(9):505-9. PMID: 2820286

Diagnosis

Mikaeel RR, Young JP, Tapia Rico G, Hewett PJ, Hardingham JE, Uylaki W, Horsnell M, Price TJ
Crit Rev Clin Lab Sci 2021 Sep;58(6):369-384. Epub 2021 Feb 11 doi: 10.1080/10408363.2021.1881756. PMID: 33569997
Holt N, Grønbæk H
ScientificWorldJournal 2013;2013:543696. Epub 2013 Jan 14 doi: 10.1155/2013/543696. PMID: 23365545Free PMC Article
Boudreaux JP, Klimstra DS, Hassan MM, Woltering EA, Jensen RT, Goldsmith SJ, Nutting C, Bushnell DL, Caplin ME, Yao JC; North American Neuroendocrine Tumor Society (NANETS)
Pancreas 2010 Aug;39(6):753-66. doi: 10.1097/MPA.0b013e3181ebb2a5. PMID: 20664473
Deschamps L, Couvelard A
Arch Pathol Lab Med 2010 Jun;134(6):871-5. doi: 10.5858/134.6.871. PMID: 20524865
Carr NJ, Sobin LH
Semin Diagn Pathol 1996 Nov;13(4):314-25. PMID: 8946609

Therapy

McConnell YJ, Mack LA, Gui X, Carr NJ, Sideris L, Temple WJ, Dubé P, Chandrakumaran K, Moran BJ, Cecil TD
Ann Surg Oncol 2014 Jun;21(6):1975-82. Epub 2014 Jan 8 doi: 10.1245/s10434-013-3469-5. PMID: 24398544

Prognosis

Madani A, van der Bilt JD, Consten EC, Vriens MR, Borel Rinkes IH
Ann Surg Oncol 2015 Mar;22(3):959-65. Epub 2014 Sep 5 doi: 10.1245/s10434-014-4023-9. PMID: 25190118
Holt N, Grønbæk H
ScientificWorldJournal 2013;2013:543696. Epub 2013 Jan 14 doi: 10.1155/2013/543696. PMID: 23365545Free PMC Article
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
Deschamps L, Couvelard A
Arch Pathol Lab Med 2010 Jun;134(6):871-5. doi: 10.5858/134.6.871. PMID: 20524865
Carr NJ, Sobin LH
Semin Diagn Pathol 1996 Nov;13(4):314-25. PMID: 8946609

Clinical prediction guides

Maedler C, Arnason T, Dorreen A, Sapp H, Castonguay M, Murphy J, Selegean S, Huang WY
Ann Diagn Pathol 2018 Feb;32:51-55. Epub 2017 Nov 7 doi: 10.1016/j.anndiagpath.2017.11.001. PMID: 29414399
Gui X, Meng Z, McConnell YJ, Liu S, Falck VG, Mack LA, Temple WJ
J Clin Pathol 2017 Jan;70(1):40-50. Epub 2016 Jul 1 doi: 10.1136/jclinpath-2016-203645. PMID: 27371613
Kang GH, Lee BS, Kang DY
Pathol Int 2015 Jun;65(6):324-8. Epub 2015 Mar 24 doi: 10.1111/pin.12284. PMID: 25808197
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
Wang HL, Dhall D
Adv Anat Pathol 2009 Jul;16(4):247-54. doi: 10.1097/PAP.0b013e3181a9d49a. PMID: 19546612

Recent systematic reviews

Madani A, van der Bilt JD, Consten EC, Vriens MR, Borel Rinkes IH
Ann Surg Oncol 2015 Mar;22(3):959-65. Epub 2014 Sep 5 doi: 10.1245/s10434-014-4023-9. PMID: 25190118

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