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Author Notes:

Correspondence: Walid Shaib, MD, Department of Hematology and Oncology, Division of GI Oncology, Winship Cancer Institute, Emory University, 1365 Clifton Rd NE, Atlanta, GA 30322, USA, Tel: 1-404-778-1900 Fax: 1-404-778-5520 E-mail: wshaib@emory.edu

Conflict of interest relevant to this article was not reported.



  • Life Sciences & Biomedicine
  • Oncology
  • Appendiceal neoplasms
  • Neuroendocrine tumors
  • Goblet cells
  • Signet ring
  • Survival
  • Disease management

Appendiceal Neuroendocrine, Goblet and Signet-Ring Cell Tumors: A Spectrum of Diseases with Different Patterns of Presentation and Outcome

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Journal Title:

Cancer Research and Treatment


Volume 48, Number 2


, Pages 596-604

Type of Work:

Article | Final Publisher PDF


Purpose Appendiceal tumors are a heterogeneous group of diseases that include typical neuroendocrine tumors (TNET), goblet cell carcinoids (GCC), and atypical GCC. Atypical GCC are classified into signet-ring cell cancers (SRCC) and poorly differentiated appendiceal adenocarcinoids. The prognosis and management of these diseases is unclear because there are no prospective studies. The aim of this study is to assess the characteristics and outcome of appendiceal TNET, GCC, and SRCC patients. Materials and Methods Appendiceal TNET, GCC, and SRCC patients diagnosed between 1973 and 2011 were identified in the Surveillance Epidemiology and End Results (SEER) database. Demographics, type of surgery, and clinicopathologic characteristics were collected. Survival functions were estimated by the Kaplan-Meier method, and log-rank test was used to assess the difference in overall survival (OS) among the three histologies. Results The SEER database yielded 1,021 TNET patients, 1,582 with GCC, and 534 SRCC patients. TNET presented at a younger age (p < 0.001). Patients with SRCC presented with advanced stage disease (p < 0.001). The median OS (mOS) for GCC and TNET patients was not reached; mOS for SRCC was 24 months. Multivariate analysis stratified for stage revealed significantly longer survival for TNET and GCC than SRCC (p < 0.001). Conclusion This is the largest report to date for appendiceal neuroendocrine tumor patients, suggesting a spectrum of diseases with different characteristics and outcomes. In this report, we present a treatment approach for this complex spectrum of disease, based on the experience of Ohio State and Emory Universities investigators.

Copyright information:

© 2016 by the Korean Cancer Association.

This is an Open Access work distributed under the terms of the Creative Commons Attribution-Noncommercial 3.0 Unported License (http://creativecommons.org/licenses/by-nc/3.0/).

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