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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

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Kieran Palmer, Scott Weerasuriya, Kandiah Chandrakumaran, Brian Rous, Benjamin E. White, Sangeeta Paisey, Rajaventhan Srirajaskanthan, John K. Ramage

Original languageEnglish
Article number915028
JournalFrontiers in oncology
Published12 Jul 2022

Bibliographical note

Funding Information: We would like to thank NHS Digital and The National Cancer Registration and Analysis Service for providing us with the registry data for analysis. This work uses data provided by patients and collected by the NHS as part of their care and support. We would also like to thank Hampshire Hospitals NHS Foundation Trust for sponsoring the study. Publisher Copyright: Copyright © 2022 Palmer, Weerasuriya, Chandrakumaran, Rous, White, Paisey, Srirajaskanthan and Ramage.

King's Authors


Background: 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. Methods: 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–Meier estimator was used to calculate overall survival, and Cox proportional hazards regression was used to identify prognostic factors. Results: The 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. Conclusions: 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.

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