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Appendiceal goblet cell carcinomas have poor survival despite completion surgery

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Abstract

Purpose

Appendiceal goblet cell carcinomas (aGCCs) are rare but aggressive tumours associated with significant mortality. We retrospectively reviewed the outcomes of aGCC patients treated at our tertiary referral centre.

Methods

We analysed aGCC patients, diagnosed between 1990–2016, assessing the impact of completion surgery and tumour factors on survival. Survival was assessed using Kaplan–Meier analysis.

Results

We identified 41 patients (23 F, 18 M); median age 61 (range 27–79) years. Mean tumour size was 10.5 (range 0.5–50) mm; most tumours were located in the appendiceal tip (n = 18, 45%). Appendicectomy was the index surgery in 32 patients, 24 of whom subsequently underwent completion surgery at median 3 (range 1.3–13.3) months later. Histology from completion surgery showed residual disease in 8 patients: nodal disease (n = 2) or residual tumour (n = 6). Index surgery for the rest was either colectomy (n = 7) or cytoreductive surgery plus intraperitoneal chemotherapy (CRS-HIPEC) (n = 1). Index and completion surgery had 0% mortality and 2.5% morbidity. Overall and recurrence-free survival were not significantly affected by tumour grade or completion surgery. Disease recurred in 9 patients after a median follow-up of 57.0 (4.6–114.9) months; 7 of these patients died during follow-up. Recurrences were treated with CRS-HIPEC (n = 1), palliative chemotherapy (n = 3) or supportive care (n = 5). Five- and ten- year overall survival were 85.3% and 62.3% respectively; 5-year and 10-year recurrence-free survival were 73.6% and 50.6%.

Conclusion

The prognosis of aGCCs remains relatively poor. Completion surgery did not prevent recurrence or improve survival, but this needs to be verified with a larger patient cohort. The high mortality associated with tumour recurrence questions current treatment recommendations.

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Acknowledgements

We would like to acknowledge Gail McKane and Rebecca Haynes for their invaluable help in collecting patient data.

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Authors and Affiliations

  1. Department of Hepatobiliary and Pancreatic Surgery, Queen’s Medical Centre, Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom

    Edward Alabraba

  2. ENETS Centre of Excellence, Liverpool University Hospitals NHS Foundation Trust, Liverpool, United Kingdom

    David Mark Pritchard, Rafael Diaz-Nieto, Melissa Banks, Daniel James Cuthbertson & Stephen Fenwick

  3. Department of Molecular and Clinical Cancer Medicine; Institute of Systems, Molecular and Integrative Biology, University of Liverpool, Liverpool, United Kingdom

    David Mark Pritchard

  4. Liverpool Clinical and Cancer Research UK Trials Unit, University of Liverpool, Liverpool, United Kingdom

    Rebecca Griffin

  5. Department of Cardiovascular and Metabolic Medicine, Institute of Life Course and Medical Sciences, University of Liverpool, Liverpool, United Kingdom

    Daniel James Cuthbertson

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Contributions

All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by EA, RG, and MB. The first draught of the manuscript was written by EA, DMP, RD-N, DJC, and SF. All authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

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Correspondence to Edward Alabraba.

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Alabraba, E., Pritchard, D.M., Griffin, R. et al. Appendiceal goblet cell carcinomas have poor survival despite completion surgery. Endocrine 73, 734–744 (2021). https://doi.org/10.1007/s12020-021-02727-9

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  • DOI: https://doi.org/10.1007/s12020-021-02727-9

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