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Journal of Medical Case Reports
Published in: Journal of Medical Case Reports 1/2018

Open Access 01-12-2018 | Case report

Goblet cell carcinoid of the appendix – diagnostic challenges and treatment updates: a case report and review of the literature

Authors: Gregory Gilmore, Kristin Jensen, Shreyas Saligram, Thomas P. Sachdev, Subramanyeswara R. Arekapudi

Published in: Journal of Medical Case Reports | Issue 1/2018

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Abstract

Background

Goblet cell carcinoid is a rare but distinct entity of appendiceal tumors which is a hybrid or mixed tumor consisting of both epithelial (glandular) and neuroendocrine elements containing goblet cells. This entity is important to recognize and appropriately grade as it tends to be more aggressive than typical carcinoid tumors, often presenting with metastatic disease. As a result, the 5-year overall survival is 14–22% in stage III–IV disease. GCC therefore warrants more aggressive surgical and medical (chemotherapy) interventions than typical carcinoid tumors. Through this case report we give a brief update on GCC pathological features, staging, surgical management, and review the literature as a guide to indications for chemotherapy and choice of agents.

Case presentation

We present the case of a 77-year-old Caucasian man with a history of stage I adenocarcinoma of transverse colon status post transverse colectomy who was incidentally found on surveillance colonoscopy to have an abnormal appendiceal orifice lesion. A biopsy revealed an appendiceal goblet cell carcinoid and he underwent a right hemicolectomy which revealed a pathologic stage III GCC for which he received eight cycles of adjuvant chemotherapy with capecitabine.

Conclusions

It is essential that patients who have tumors > 2 cm, are pT3 or pT4, have higher grade histology with signet ring (Tang grade B or grade C), locally advanced, or with positive surgical margins on appendectomy undergo a right hemicolectomy. Although there is no category 1 evidence, consensus recommendations are that patients with stage II (particularly Tang B and C) and stage III GCC be offered adjuvant chemotherapy with a regimen based on 5-fluorouracil, as these patients are known to have high rates of relapse.
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Metadata
Title
Goblet cell carcinoid of the appendix – diagnostic challenges and treatment updates: a case report and review of the literature
Authors
Gregory Gilmore
Kristin Jensen
Shreyas Saligram
Thomas P. Sachdev
Subramanyeswara R. Arekapudi
Publication date
01-12-2018
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2018
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-018-1789-6

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