Copyright ©The Author(s) 2016. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Oct 27, 2016; 8(10): 660-669
Published online Oct 27, 2016. doi: 10.4240/wjgs.v8.i10.660
Goblet cell carcinoids of the appendix: Tumor biology, mutations and management strategies
Santosh Shenoy
Santosh Shenoy, Department of Surgery, KCVA and University of Missouri Kansas City, Kansas City, MO 64128, United States
Author contributions: Shenoy S collected the data, wrote, and revised the manuscript.
Conflict-of-interest statement: There is no conflict of interest, no funding or financial disclosures.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Correspondence to: Santosh Shenoy, MD, FACS, Department of Surgery, KCVA and University of Missouri Kansas City, 4801 E Linwood Blvd, Kansas City, MO 64128, United States.
Telephone: +1-816-8614700-55431
Received: June 17, 2016
Peer-review started: June 18, 2016
First decision: July 27, 2016
Revised: August 14, 2016
Accepted: August 27, 2016
Article in press: August 29, 2016
Published online: October 27, 2016
Core Tip

Core tip: Goblet cell carcinoids tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features. The pathogenesis is unclear however the tumor likely arises from pluripotent intestinal epithelial crypt base stem cells. Loss of Notch signaling may be the driver mutation with other successive downstream mutations likely favors them into progressing and behavior similar to poorly differentiated adenocarcinoma with minimal neuroendocrine differentiation. Surgery remains the main treatment modality. We discuss the clinical implications of this cancer focusing on the tumor biology, mutations, signaling mechanisms and management.