Appendiceal goblet cell carcinomas have poor survival despite completion surgery

Alabraba, Edward, Pritchard, David Mark ORCID: 0000-0001-7971-3561, Griffin, Rebecca ORCID: 0000-0003-4521-948X, Diaz-Nieto, Rafael, Banks, Melissa, Cuthbertson, Daniel James ORCID: 0000-0002-6128-0822 and Fenwick, Stephen
(2021) Appendiceal goblet cell carcinomas have poor survival despite completion surgery. ENDOCRINE, 73 (3). pp. 734-744.

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<h4>Purpose</h4>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.<h4>Methods</h4>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.<h4>Results</h4>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%.<h4>Conclusion</h4>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.

Item Type: Article
Uncontrolled Keywords: Appendiceal, Appendix, Completion right hemicolectomy, Completion surgery, Goblet cell carcinoids, Goblet cell carcinomas
Divisions: Faculty of Health and Life Sciences
Faculty of Health and Life Sciences > Institute of Systems, Molecular and Integrative Biology
Depositing User: Symplectic Admin
Date Deposited: 29 Apr 2021 08:36
Last Modified: 08 Mar 2023 09:56
DOI: 10.1007/s12020-021-02727-9
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