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Appendix neuroendocrine tumor G1

MedGen UID:
90758
•Concept ID:
C0334298
•
Neoplastic Process
Synonyms: Appendiceal Carcinoid Tumor; appendiceal carcinoid tumor; appendiceal carcinoid tumour; appendix carcinoid endocrine tumor; appendix carcinoid endocrine tumour; Appendix Carcinoid Tumor; appendix carcinoid tumor; appendix carcinoid tumour; Appendix NET G1; appendix NET G1; Appendix NET G1 (Carcinoid); appendix NET G1 (carcinoid); Appendix Neuroendocrine Tumor G1; appendix neuroendocrine tumor G1; Appendix Neuroendocrine Tumor G1 (Carcinoid); appendix neuroendocrine tumor G1 (carcinoid); appendix neuroendocrine tumour G1 (carcinoid); Carcinoid of appendix; Carcinoid tumor of appendix; Carcinoid Tumor of Appendix; carcinoid tumor of appendix; Carcinoid Tumor of the Appendix; carcinoid tumor of the appendix; Carcinoid tumor of uncertain malignant potential; Carcinoid tumor, argentaffin; Carcinoid tumor, of appendix; carcinoid tumour of appendix; carcinoid tumour of the appendix; Carcinoid, of appendix; grade 1 neuroendocrine neoplasm of vermiform appendix; vermiform appendix carcinoid tumor; vermiform appendix carcinoid tumor (disease); vermiform appendix carcinoid tumour; vermiform appendix carcinoid tumour (disease); vermiform appendix NET G1; vermiform appendix neuroendocrine neoplasm G1; vermiform appendix neuroendocrine tumor, well differentiated, low grade
SNOMED CT: Carcinoid tumor, argentaffin (22228003); Carcinoid tumor of uncertain malignant potential (22228003); Carcinoid tumor, of appendix (22228003); Carcinoid, of appendix (22228003); Carcinoid tumor of appendix (253002004); Carcinoid of appendix (253002004)
 
Monarch Initiative: MONDO:0006091

Definition

A well differentiated, low grade neoplasm with neuroendocrine differentiation that arises from the appendix. The mitotic count is less than 2 per 10 HPF and/or the Ki67 index is equal to or less than 2 percent. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAppendix neuroendocrine tumor G1

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
Gastro-entero-pancreatic neuroendocrine neoplasia: The rules for non-operative management.
Panzuto F, Massironi S, Partelli S, Campana D, Rinzivillo M, Invernizzi P, Andreasi V, Lamberti G, Falconi M
Surg Oncol 2020 Dec;35:141-148. Epub 2020 Aug 20 doi: 10.1016/j.suronc.2020.08.015. PMID: 32877883

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
Well-Differentiated Neuroendocrine Tumors of the Appendix in Children and Adolescents: A Clinicopathologic Study.
Simon CT, Ehrlich P, Hryhorczuk A, Rabah R, Sedig L, Stoll T, Heider A
Pediatr Dev Pathol 2023 May-Jun;26(3):250-258. Epub 2023 Feb 21 doi: 10.1177/10935266221146001. PMID: 37334832
Lights and darks of neuroendocrine tumors of the appendix.
Tamagno G, Bennett A, Ivanovski I
Minerva Endocrinol 2020 Dec;45(4):381-392. Epub 2020 Jul 23 doi: 10.23736/S0391-1977.20.03206-X. PMID: 32720498
Prognostic factors of patients with gastroenteropancreatic neuroendocrine neoplasms.
Huang PY, Tsai KL, Liang CM, Tai WC, Rau KM, Wu KL, Huang CC, Chuah SK
Kaohsiung J Med Sci 2018 Nov;34(11):650-656. Epub 2018 Jun 20 doi: 10.1016/j.kjms.2018.05.009. PMID: 30392572
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

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
Well-Differentiated Neuroendocrine Tumors of the Appendix in Children and Adolescents: A Clinicopathologic Study.
Simon CT, Ehrlich P, Hryhorczuk A, Rabah R, Sedig L, Stoll T, Heider A
Pediatr Dev Pathol 2023 May-Jun;26(3):250-258. Epub 2023 Feb 21 doi: 10.1177/10935266221146001. PMID: 37334832
Lights and darks of neuroendocrine tumors of the appendix.
Tamagno G, Bennett A, Ivanovski I
Minerva Endocrinol 2020 Dec;45(4):381-392. Epub 2020 Jul 23 doi: 10.23736/S0391-1977.20.03206-X. PMID: 32720498
Prognostic factors of patients with gastroenteropancreatic neuroendocrine neoplasms.
Huang PY, Tsai KL, Liang CM, Tai WC, Rau KM, Wu KL, Huang CC, Chuah SK
Kaohsiung J Med Sci 2018 Nov;34(11):650-656. Epub 2018 Jun 20 doi: 10.1016/j.kjms.2018.05.009. PMID: 30392572
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

Therapy

Treatment sequence in patients with neuroendocrine tumours: a nationwide multicentre, observational analysis of the Swiss neuroendocrine tumour registry.
Kollar A, Bütikofer L, Ochsenbein A, Stettler C, Trepp R
Swiss Med Wkly 2020 Jan 15;150:w20176. doi: 10.4414/smw.2020.20176. PMID: 31940430
Clinicopathologic characteristics and prognosis of gastroenteropancreatic neuroendocrine neoplasms: a multicenter study in South China.
Fang C, Wang W, Zhang Y, Feng X, Sun J, Zeng Y, Chen Y, Li Y, Chen M, Zhou Z, Chen J
Chin J Cancer 2017 Jun 21;36(1):51. doi: 10.1186/s40880-017-0218-3. PMID: 28637502Free PMC Article
Digestive Neuroendocrine Tumor Distribution and Characteristics According to the 2010 WHO Classification: a Single Institution Experience in Lebanon.
Kourie HR, Ghorra C, Rassy M, Kesserouani C, Kattan J
Asian Pac J Cancer Prev 2016;17(5):2679-81. PMID: 27268650
Identification of the BRAF V600E mutation in gastroenteropancreatic neuroendocrine tumors.
Park C, Ha SY, Kim ST, Kim HC, Heo JS, Park YS, Lauwers G, Lee J, Kim KM
Oncotarget 2016 Jan 26;7(4):4024-35. doi: 10.18632/oncotarget.6602. PMID: 26684240Free PMC Article

Prognosis

Well-Differentiated Neuroendocrine Tumors of the Appendix in Children and Adolescents: A Clinicopathologic Study.
Simon CT, Ehrlich P, Hryhorczuk A, Rabah R, Sedig L, Stoll T, Heider A
Pediatr Dev Pathol 2023 May-Jun;26(3):250-258. Epub 2023 Feb 21 doi: 10.1177/10935266221146001. PMID: 37334832
Gastro-entero-pancreatic neuroendocrine neoplasia: The rules for non-operative management.
Panzuto F, Massironi S, Partelli S, Campana D, Rinzivillo M, Invernizzi P, Andreasi V, Lamberti G, Falconi M
Surg Oncol 2020 Dec;35:141-148. Epub 2020 Aug 20 doi: 10.1016/j.suronc.2020.08.015. PMID: 32877883
Lights and darks of neuroendocrine tumors of the appendix.
Tamagno G, Bennett A, Ivanovski I
Minerva Endocrinol 2020 Dec;45(4):381-392. Epub 2020 Jul 23 doi: 10.23736/S0391-1977.20.03206-X. PMID: 32720498
Prognostic factors of patients with gastroenteropancreatic neuroendocrine neoplasms.
Huang PY, Tsai KL, Liang CM, Tai WC, Rau KM, Wu KL, Huang CC, Chuah SK
Kaohsiung J Med Sci 2018 Nov;34(11):650-656. Epub 2018 Jun 20 doi: 10.1016/j.kjms.2018.05.009. PMID: 30392572
Clinicopathologic characteristics and prognosis of gastroenteropancreatic neuroendocrine neoplasms: a multicenter study in South China.
Fang C, Wang W, Zhang Y, Feng X, Sun J, Zeng Y, Chen Y, Li Y, Chen M, Zhou Z, Chen J
Chin J Cancer 2017 Jun 21;36(1):51. doi: 10.1186/s40880-017-0218-3. PMID: 28637502Free PMC Article

Clinical prediction guides

A multigenomic liquid biopsy biomarker for neuroendocrine tumor disease outperforms CgA and has surgical and clinical utility.
Modlin IM, Kidd M, Falconi M, Filosso PL, Frilling A, Malczewska A, Toumpanakis C, Valk G, Pacak K, Bodei L, Öberg KE
Ann Oncol 2021 Nov;32(11):1425-1433. Epub 2021 Aug 11 doi: 10.1016/j.annonc.2021.08.1746. PMID: 34390828Free PMC Article
Gastrointestinal neuro-endocrine tumors: retrospective study of 36 cases.
Zehani A, Aloui S, Chelly I, Haouet S, Kchir N
Tunis Med 2017 Jun;95(6):393-400. PMID: 29512793
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
Clinicopathologic characteristics and prognosis of gastroenteropancreatic neuroendocrine neoplasms: a multicenter study in South China.
Fang C, Wang W, Zhang Y, Feng X, Sun J, Zeng Y, Chen Y, Li Y, Chen M, Zhou Z, Chen J
Chin J Cancer 2017 Jun 21;36(1):51. doi: 10.1186/s40880-017-0218-3. PMID: 28637502Free PMC Article
Acute appendicitis with a neuroendocrine tumor G1 (carcinoid): pitfalls of conservative treatment.
Watanabe HA, Fujimoto T, Kato Y, Sasaki M, Ikusue T
Clin J Gastroenterol 2016 Aug;9(4):203-7. Epub 2016 Jun 16 doi: 10.1007/s12328-016-0660-9. PMID: 27311320

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