Goblet cell carcinoid of the appendix and mixed adenoneuroendocrine carcinoma: Report of three cases

Karaman, Hati̇ce; Şenel, Fatma; Güreli, Mustafa; Ekinci, Turan; Topuz, Ömer

doi:10.4251/wjgo.v9.i7.308

Cited by 5 publications

(7 citation statements)

References 18 publications

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“…The most common presenting feature of GCA is acute appendicitis ( 34 , 39 , 40 , 42 , 54 , 56 , 66 , 68 , 72 , 77 , 97 , 99 , 102 ), followed by non-specific abdominal pain or an abdominal mass ( 4 , 28 , 44 , 52 , 58 , 62 , 65 , 71 , 73 , 80 , 81 , 98 ). Appendicitis is common in low-grade and localised disease ( 7 , 52 , 55 , 89 ) and non-specific abdominal pain with or without abdominal mass in higher-grade or metastatic disease ( 7 ).…”

Section: Resultsmentioning

confidence: 99%

“…Our review included 104 studies (Figure 1). These consisted of one meta-analysis (8), one systematic review (9), one prospective cohort study (10), 18 registry studies (2,3,(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26), 66 single or multicentre retrospective analyses (4,7,, four inter-user variability studies (91)(92)(93)(94), and 13 case series (95)(96)(97)(98)(99)(100)(101)(102)(103)(104)(105)(106)(107). A quantitative meta-analysis of the studies was precluded by significant heterogeneity in the nomenclature and data.…”

Section: Systematic Reviewmentioning

confidence: 99%

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Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

et al. 2022

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BackgroundGoblet cell adenocarcinoma (GCA) of the appendix is a rare and aggressive tumour with varying nomenclature and classification systems. This has led to heterogeneity in published data, and there is a lack of consensus on incidence, survival, and management.MethodsWe provide an overview of GCA with a comprehensive systematic review using Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology and a retrospective analysis of all cases recorded in the English National Cancer Registration and Analysis Service database between 1995 and 2018. The Kaplan–Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors.ResultsThe systematic review demonstrated an incidence of 0.05–0.3 per 100,000 per year among North American registry studies. The 1-, 3-, and 5-year survival rate was 95.5%, 85.9%–87.6%, and 76.0%–80.6%, respectively. Age, stage, and grade were identified as prognostic factors for survival. Our analysis included 1,225 cases. Age-standardised incidence was 0.0335 per year in 1995 and gradually rose to 0.158 per year in 2018. The 1-, 3-, and 5-year survival rate was 90.0% [95% confidence interval (95% CI): 85.4–94.0], 76.0% (95% CI: 73.8–80.9), and 68.6% (95% CI: 65.9–72.2), respectively. On univariate Cox regression analyses, female sex, stage, and grade were associated with worse overall survival. On multivariate analysis, only stage remained a statistically significant prognostic factor.ConclusionsGCA of the appendix is rare, but incidence is increasing. We report a lower incidence and survival than North American registry studies. Higher stage was associated with decreased survival. Further prospective studies are required to establish optimal management.

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Section: Resultsmentioning

confidence: 99%

Section: Systematic Reviewmentioning

confidence: 99%

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

et al. 2022

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“…Goblet cell-type carcinomas are typically more aggressive, and signet ring cell classification is ‘defined as goblet cells or signet ring cells arranged in irregular large clusters, with the lack of confluent sheets of cells in a discohesive single file or single cell infiltrating pattern with significant cytologic atypia, and desmoplasia and associated destruction of the appendiceal wall’ [ 7 ]. The 5-year survival rate is 20.5% and will drop to 6.7–14% if there is diffuse peritoneal metastasis [ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…It is also noted that mucin droplets and neuroendocrine secretory granules are present in these epithelial intestinal crypts that help form the tumor [ 5 ]. Appendectomy is the treatment of choice followed by superimposed right hemicolectomy and adjuvant chemotherapy if warranted from perforation or invasion of location structures [ 7 ].…”

Section: Introductionmentioning

confidence: 99%

A rare case of signet ring cell carcinoma of the appendix

Caesar-Peterson

Tulla

Southall

et al. 2020

Journal of Surgical Case Reports

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Primary appendiceal carcinoma itself comprises less than 0.5% of all gastrointestinal malignant neoplasms. However, signet ring cell carcinoma of the appendix is an extremely rare subset of primary appendiceal carcinomas. This sub-type of appendiceal carcinoma represents only 4% of all appendiceal carcinomas. In this case report, we present a case of signet ring cell carcinoma found in a 65-year-old male patient which presented as acute appendicitis. He was diagnosed after a laparoscopic appendectomy by histopathological evaluation.

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“…Goblet cell carcinoids (GCC) are rare neuroendocrine tumours first described by Gagne et al in 1969. 1 They are an amphicrine tumour consisting of both epithelial (adenocarcinoma) and neuroendocrine (carcinoid) components and are grouped under the category of adenocarcinoids. 2 They virtually solely involve the appendix with some exceptional extraappendiceal locations being reported including the stomach, duodenum, small intestine, colon and rectum.…”

Section: Introductionmentioning

confidence: 99%

Extraappendiceal goblet cell carcinoid of the transverse colon

Bowles¹

2021

Int Surg J

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Goblet cell carcinoids (GCC) are a rare subgroup of neuroendocrine tumours which exhibit mixed endocrine and exocrine features and are almost exclusively localised to the appendix. A few cases of extraappendiceal GCC have been reported in the literature however these are exceedingly rare. They are biologically aggressive tumours that are believed to arise from pluripotent intestinal epithelial crypt base stem cells. Given their rarity, no guidelines currently exist for the management of extraappendiceal GCC, however treatment for GCC’s is generally based on their tumour stage in line with staging for typical adenocarcinomas. This case gives an account of a colonic goblet cell carcinoid within the transverse colon encountered in a 66-year-old male. A literature search did not identify any previous documented cases of a GCC at this location.

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Goblet cell carcinoid of the appendix and mixed adenoneuroendocrine carcinoma: Report of three cases

Cited by 5 publications

References 18 publications

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry

A rare case of signet ring cell carcinoma of the appendix

Extraappendiceal goblet cell carcinoid of the transverse colon

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