{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,22]],"date-time":"2026-10-22T05:48:42Z","timestamp":1792648122493,"version":"4.5.0"},"reference-count":114,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,7,12]],"date-time":"2022-07-12T00:00:00Z","timestamp":1657584000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Goblet 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We 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\u2013Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The systematic review demonstrated an incidence of 0.05\u20130.3 per 100,000 per year among North American registry studies. The 1-, 3-, and 5-year survival rate was 95.5%, 85.9%\u201387.6%, and 76.0%\u201380.6%, 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\u201394.0], 76.0% (95% CI: 73.8\u201380.9), and 68.6% (95% CI: 65.9\u201372.2), 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>GCA 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.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2022.915028","type":"journal-article","created":{"date-parts":[[2022,7,12]],"date-time":"2022-07-12T04:50:58Z","timestamp":1657601458000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":49,"title":["Goblet Cell Adenocarcinoma of the Appendix: A Systematic Review and Incidence and Survival of 1,225 Cases From an English Cancer Registry"],"prefix":"10.3389","volume":"12","author":[{"given":"Kieran","family":"Palmer","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott","family":"Weerasuriya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kandiah","family":"Chandrakumaran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian","family":"Rous","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benjamin E.","family":"White","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sangeeta","family":"Paisey","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajaventhan","family":"Srirajaskanthan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John K.","family":"Ramage","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,7,12]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"151401","DOI":"10.1016\/j.anndiagpath.2019.151401","article-title":"Goblet Cell Tumors of the Appendix: A Review","volume":"43","author":"Sinno","year":"2019","journal-title":"Ann Diagn Pathol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1016\/j.suronc.2020.12.003","article-title":"Is Adjuvant Chemotherapy Beneficial for Stage II-III Goblet Cell Carcinoid\/Goblet Cell Adenocarcinoma of the Appendix","volume":"36","author":"Zakka","year":"2021","journal-title":"Surg Oncol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.4143\/crt.2015.029","article-title":"Appendiceal Neuroendocrine, Goblet and Signet-Ring Cell Tumors: A Spectrum of Diseases With Different Patterns of Presentation and Outcome","volume":"48","author":"Shaib","year":"2016","journal-title":"Cancer Res Treat"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1007\/s12253-010-9291-5","article-title":"Clinicopathological Features and Immunoexpression Profiles of Goblet Cell Carcinoid and Typical Carcinoid of the Appendix","volume":"17","author":"Jiang","year":"2011","journal-title":"Pathol Oncol Res"},{"key":"B5","volume-title":"TNM Classification of Malignant Tumours","author":"Brierley","year":"2016"},{"key":"B6","doi-asserted-by":"publisher","first-page":"16","DOI":"10.1093\/ije\/dyz076","article-title":"Data Resource Profile: National Cancer Registration Dataset in England","volume":"49","author":"Henson","year":"2020","journal-title":"Int J Epidemiol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31817f1816","article-title":"Pathologic Classification and Clinical Behavior of the Spectrum of Goblet Cell Carcinoid Tumors of the Appendix","volume":"32","author":"Tang","year":"2008","journal-title":"Am J Surg Pathol"},{"key":"B8","article-title":"Adenocarcinoid of the Appendix: Is Right Hemicolectomy Necessary? 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