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Appendix Neuroendocrine Carcinoma

MedGen UID:
473544
Concept ID:
C2205024
Neoplastic Process
Synonyms: Appendix High Grade Neuroendocrine Carcinoma; Appendix NEC; Appendix NEC G3; Appendix Poorly Differentiated Endocrine Carcinoma; Neuroendocrine carcinoma of appendix
SNOMED CT: Neuroendocrine carcinoma of appendix (717916003)

Definition

An aggressive, high-grade, and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the appendix. The mitotic count is more than 20 per 10 HPF. It is classified as either small or large cell neuroendocrine carcinoma based on the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Bazarbashi S, Aseafan M, Elgazzar T, Alkhayat M, Alghabban A, Abdelgawad MI, Alshamsan B, Alshibany A, Elhassan T, Aljubran A, Alzahrani A, Alhindi H, Raef H
BMC Endocr Disord 2023 Apr 7;23(1):74. doi: 10.1186/s12902-023-01326-1. PMID: 37029347Free PMC Article
Cuthbertson DJ, Shankland R, Srirajaskanthan R
Clin Med (Lond) 2023 Mar;23(2):119-124. doi: 10.7861/clinmed.2023-0044. PMID: 36958842Free PMC Article
Niederhuber JE, Fojo T
Surg Oncol Clin N Am 2006 Jul;15(3):511-33, viii. doi: 10.1016/j.soc.2006.05.004. PMID: 16882495

Recent clinical studies

Etiology

Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Sinno SAJ, Jurdi NMH
Ann Diagn Pathol 2019 Dec;43:151401. Epub 2019 Aug 21 doi: 10.1016/j.anndiagpath.2019.151401. PMID: 31675676
Patel N, Barbieri A, Gibson J
Surg Pathol Clin 2019 Dec;12(4):1021-1044. doi: 10.1016/j.path.2019.08.007. PMID: 31672292
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

Diagnosis

Chauhan A, Chan K, Halfdanarson TR, Bellizzi AM, Rindi G, O'Toole D, Ge PS, Jain D, Dasari A, Anaya DA, Bergsland E, Mittra E, Wei AC, Hope TA, Kendi AT, Thomas SM, Flem S, Brierley J, Asare EA, Washington K, Shi C
CA Cancer J Clin 2024 Jul-Aug;74(4):359-367. Epub 2024 Apr 29 doi: 10.3322/caac.21840. PMID: 38685134
Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Cuthbertson DJ, Shankland R, Srirajaskanthan R
Clin Med (Lond) 2023 Mar;23(2):119-124. doi: 10.7861/clinmed.2023-0044. PMID: 36958842Free PMC Article
Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Sinno SAJ, Jurdi NMH
Ann Diagn Pathol 2019 Dec;43:151401. Epub 2019 Aug 21 doi: 10.1016/j.anndiagpath.2019.151401. PMID: 31675676

Therapy

Köhler F, Matthes N, Rosenfeldt M, Kunzmann V, Germer CT, Wiegering A
Dtsch Arztebl Int 2023 Aug 7;120(31-32):519-525. doi: 10.3238/arztebl.m2023.0136. PMID: 37282595Free PMC Article
Cuthbertson DJ, Shankland R, Srirajaskanthan R
Clin Med (Lond) 2023 Mar;23(2):119-124. doi: 10.7861/clinmed.2023-0044. PMID: 36958842Free PMC Article
Eftimie MA, Potlog G, Alexandrescu ST
Medicina (Kaunas) 2023 Jan 28;59(2) doi: 10.3390/medicina59020255. PMID: 36837456Free 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
Niederhuber JE, Fojo T
Surg Oncol Clin N Am 2006 Jul;15(3):511-33, viii. doi: 10.1016/j.soc.2006.05.004. PMID: 16882495

Prognosis

Volante M, Grillo F, Massa F, Maletta F, Mastracci L, Campora M, Ferro J, Vanoli A, Papotti M
Pathologica 2021 Feb;113(1):19-27. doi: 10.32074/1591-951X-230. PMID: 33686307Free PMC Article
Hrabe J
Surg Oncol Clin N Am 2020 Apr;29(2):267-279. doi: 10.1016/j.soc.2019.11.010. PMID: 32151360
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
Sinno SAJ, Jurdi NMH
Ann Diagn Pathol 2019 Dec;43:151401. Epub 2019 Aug 21 doi: 10.1016/j.anndiagpath.2019.151401. PMID: 31675676
Patel N, Barbieri A, Gibson J
Surg Pathol Clin 2019 Dec;12(4):1021-1044. doi: 10.1016/j.path.2019.08.007. PMID: 31672292

Clinical prediction guides

Lin W, Zhu Z, Shang Y
J Cancer Res Clin Oncol 2024 Jan 2;150(1):4. doi: 10.1007/s00432-023-05568-3. PMID: 38168014
Kerekes D, Frey A, Bakkila B, Kunstman JW, Khan SA
J Surg Oncol 2023 Oct;128(5):790-802. Epub 2023 Jul 12 doi: 10.1002/jso.27392. PMID: 37435780
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
Clift AK, Frilling A
Expert Rev Gastroenterol Hepatol 2017 Mar;11(3):237-247. Epub 2017 Jan 20 doi: 10.1080/17474124.2017.1282314. PMID: 28081662
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

Recent systematic reviews

Grossi U, Bonis A, Carrington EV, Mazzobel E, Santoro GA, Cattaneo L, Centonze G, Gallo G, Kazemi Nava A, Romano M, Di Tanna GL, Zanus G
Eur J Surg Oncol 2021 Nov;47(11):2893-2899. Epub 2021 May 14 doi: 10.1016/j.ejso.2021.05.021. PMID: 34052038
Machairas N, Paspala A, Frountzas M, Tsilimigras DI, Moris D, Ntomi V, Tsapralis D, Schizas D
In Vivo 2019 Nov-Dec;33(6):1721-1726. doi: 10.21873/invivo.11662. PMID: 31662496Free PMC Article

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