Editorial
Copyright ©2010 Baishideng. All rights reserved.
World J Gastrointest Oncol. Jun 15, 2010; 2(6): 251-258
Published online Jun 15, 2010. doi: 10.4251/wjgo.v2.i6.251
Goblet cell carcinoid tumors of the appendix: An overview
Paromita Roy, Runjan Chetty
Paromita Roy, Runjan Chetty, Department of Pathology, Laboratory Medicine Programme, University of Toronto, Toronto, M5G2C4, Canada
Runjan Chetty, Department of Pathology, University of Glasgow, Glasgow, Scotland, G11 6NT, United Kingdom
Author contributions: Roy P collected the material, prepared the manuscript and approved the final version; Chetty R prepared the manuscript and approved the final version.
Correspondence to: Runjan Chetty, Professor, Department of Pathology, University of Glasgow, McGregor Building, The Western Infirmary, Dumbarton Road, Glasgow, Scotland, G11 6NT, United Kingdom. runjan.chetty@gmail.com
Telephone: +44-141-2112233 Fax: +44-141-3372494
Received: December 14, 2009
Revised: January 16, 2010
Accepted: January 23, 2010
Published online: June 15, 2010
Abstract

Goblet cell carcinoid is an enigmatic and rare tumor involving the appendix almost exclusively. Since its identification in 1969, understanding of this disease has evolved greatly, but issues regarding its histogenesis, nomenclature and management are still conjectural. The published English language literature from 1966 to 2009 was retrieved via PubMed and reviewed. Various other names have been used for this entity such as adenocarcinoid, mucinous carcinoid, crypt cell carcinoma, and mucin-producing neuroendocrine tumor, although none have been found to be completely satisfactory or universally accepted. The tumor is thought to arise from pluripotent intestinal epithelial crypt-base stem cells by dual neuroendocrine and mucinous differentiation. GCCs present in the fifth to sixth decade and show no definite sex predominance. The most common clinical presentation is acute appendicitis, followed by abdominal pain and a mass. Fifty percent of the female patients present with ovarian metastases. The histologic hallmark of this entity is the presence of clusters of goblet cells in the lamina propria or submucosa stain for various neuroendocrine markers, though the intensity is often patchy. Atypia is usually minimal, but carcinomatous growth patterns may be seen. These may be of signet ring cell type or poorly differentiated adenocarcinoma. Recently molecular studies have shown these tumors to lack the signatures of adenocarcinoma but they have some changes similar to that of ileal carcinoids (allelic loss of chromosome 11q, 16q and 18q). The natural history of GCC is intermediate between carcinoids and adenocarcinomas of the appendix. The 5-year overall survival is 76%. The most important prognostic factor is the stage of disease. Appendectomy and right hemicolectomy are the main modalities of treatment, followed by adjuvant chemotherapy in select cases. There is some debate about the surgical approach for these tumors, and a summary of published series and recommendations are provided.

Keywords: Goblet cell carcinoid; Appendiceal neoplasm; Mucin-producing neuroendocrine tumor of appendix