Goblet cell carcinomas of the appendix: rare but aggressive neoplasms with challenging management.
File(s)
Published version
Author(s)
Type
Journal Article
Abstract
Goblet cell carcinomas (GCC) are a rare, aggressive sub-type of appendiceal tumours with neuroendocrine features, and controversy exists with regards to therapeutic strategy. We undertook a retrospective review of GCC patients surgically treated at two tertiary referral centres. Clinical and histopathological data were extracted from a prospectively maintained database. Survival analyses utilised Kaplan-Meier methodology. Twenty-one patients were identified (9 females). Median age at diagnosis was 55years (range 32-77). There were 3, 6 and 9 grade 1, 2 and 3 tumours, respectively. One, 10, 5 and 5 patients had stage I, II, III and IV disease at diagnosis, respectively. There were 8, 10 and 3 Tang class A, B and C tumours, respectively. Index operation was appendectomy (n=12), right hemicolectomy (n=6) or resections including appendix/right colon, omentum and the gynaecological system (n=3). Eight patients underwent completion right hemicolectomy. Surgery for recurrence included small bowel resection (n=2), debulking with peritonectomy and heated intraperitoneal chemotherapy, and hysterectomy and bilateral salpingo-oophorectomy (all n=1). Median follow-up was 30months (range 2.5-123). One-, 3- and 5-year OS was 79.4%, 60% and 60%, respectively. Mean OS (1-, 3-, and 5-year OS) for Tang class A, B and C tumours were 73.1months (85.7%, 85.7%, 51.4%), 83.7months (all 66.7%) and 28.5months (66.7%, 66.7%, not reached), respectively. Chromogranin A/B and 68Ga-DOTATATE PET/CT were not useful in follow-up, but CEA, CA 19-9, CA 125 and 18F-FDG PET/CT identified tumour recurrence. GCC must be clearly discriminated from relatively indolent appendiceal neuroendocrine neoplasms. 18F-FDG PET/CT and CEA/CA19-9/CA-125 are useful in detecting recurrence of GCC.
Date Issued
2018-01-15
Date Acceptance
2018-01-15
Citation
Endocrine Connections, 2018, 7 (2), pp.268-277
ISSN
2049-3614
Publisher
BioScientifica
Start Page
268
End Page
277
Journal / Book Title
Endocrine Connections
Volume
7
Issue
2
Copyright Statement
This work is licensed under a Creative Commons
Attribution-NonCommercial-NoDerivatives 4.0
International License.
Attribution-NonCommercial-NoDerivatives 4.0
International License.
Sponsor
Dr. Heinz-Horst Deichmann Stiftung
Identifier
PII: EC-17-0311
Grant Number
n/a
Subjects
appendix
goblet cell carcinoma
neoplasm
neuroendocrine tumours
Publication Status
Published online