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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

Characteristics and treatment results of patients with gastroenteropancreatic neuroendocrine tumors in a tertiary care centre.
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
Diagnosis and management of neuroendocrine tumours.
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
Consensus guidelines for the management of patients with digestive neuroendocrine tumours: well-differentiated tumour/carcinoma of the appendix and goblet cell carcinoma.
Plöckinger U, Couvelard A, Falconi M, Sundin A, Salazar R, Christ E, de Herder WW, Gross D, Knapp WH, Knigge UP, Kulke MH, Pape UF; Frascati Consensus Conference participants
Neuroendocrinology 2008;87(1):20-30. Epub 2007 Oct 11 doi: 10.1159/000109876. PMID: 17934252

Recent clinical studies

Etiology

Well-differentiated neuroendocrine tumors of the appendix: Diagnosis, differentials, and disease progression.
Navale P, Gonzalez RS
Semin Diagn Pathol 2024 Sep;41(5):236-242. Epub 2024 Aug 14 doi: 10.1053/j.semdp.2024.08.001. PMID: 39168792
Neoplasms of the Appendix.
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
Clinical Analysis of Extrapulmonary Neuroendocrine Carcinoma: A Retrospective and Single Institution Experience.
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
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
Neuroendocrine Tumors of the Gastrointestinal Tract and Pancreas.
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

Diagnosis

Critical updates in neuroendocrine tumors: Version 9 American Joint Committee on Cancer staging system for gastroenteropancreatic neuroendocrine tumors.
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
Neoplasms of the Appendix.
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
Diagnosis and management of neuroendocrine tumours.
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
Clinical Analysis of Extrapulmonary Neuroendocrine Carcinoma: A Retrospective and Single Institution Experience.
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
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

Therapy

Neoplasms of the Appendix.
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
Diagnosis and management of neuroendocrine tumours.
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
Appendix 2: Medullary Thyroid Cancer: eUpdate published online 19 February 2018  (http://www.esmo.org/Guidelines/Endocrine-and-Neuroendocrine-Cancers/Thyroid-cancer).
ESMO Guidelines Committee
Ann Oncol 2018 Oct 1;29(Suppl 4):iv257-iv258. doi: 10.1093/annonc/mdy165. PMID: 30285217
Effects of Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy (HIPEC) in the Treatment of Goblet Cell Carcinoma: A Prospective Cohort Study.
Madsen AH, Ladekarl M, Villadsen GE, Grønbæk H, Sørensen MM, Stribolt K, Verwaal VJ, Iversen LH
Ann Surg Oncol 2018 Feb;25(2):422-430. Epub 2017 Dec 6 doi: 10.1245/s10434-017-6272-x. PMID: 29214450
Everolimus and pancreatic neuroendocrine tumors (PNETs): Activity, resistance and how to overcome it.
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

Prognosis

Clinical Analysis of Extrapulmonary Neuroendocrine Carcinoma: A Retrospective and Single Institution Experience.
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
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
Neuroendocrine neoplasms of the appendix, colon and rectum.
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
Neuroendocrine Tumors of the Appendix, Colon, and Rectum.
Hrabe J
Surg Oncol Clin N Am 2020 Apr;29(2):267-279. doi: 10.1016/j.soc.2019.11.010. PMID: 32151360
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

Clinical prediction guides

Gastroenteropancreatic neuroendocrine carcinoma in children and adolescents: a population-based study.
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
Mixed adenoneuroendocrine carcinoma originating from the appendix and colorectum: a comparative analysis of a large population-based database.
Zhao B, Wang J, Ye H, Cheng X, Meng K, Yang T
Tech Coloproctol 2023 Nov;27(11):1017-1024. Epub 2023 Mar 30 doi: 10.1007/s10151-023-02786-2. PMID: 36995458
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
Neuroendocrine, goblet cell and mixed adeno-neuroendocrine tumours of the appendix: updates, clinical applications and the future.
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
Pathology of gastrointestinal disorders.
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

Mixed adenoneuroendocrine carcinoma (MANEC) of the lower gastrointestinal tract: A systematic review with Bayesian hierarchical survival analysis.
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
Mixed Adenoneuroendocrine Carcinoma (MANEC) of the Gallbladder: A Systematic Review of Outcomes Following Surgical Management.
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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