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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: http://creativecommons.org/licenses/by-nc/4.0/
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. shenoy2009@hotmail.com
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
Processing time: 130 Days and 10.7 Hours
Abstract

Malignant neoplasms of the appendix are rare and represent less than 1% of gastrointestinal cancers. Goblet cell carcinoids (GCC) tumors are a distinctive group of heterogeneous appendiceal neoplasm that exhibit unique clinical and pathologic features. This review focuses on the current diagnostic procedures, pathogenesis, possible signaling mechanisms and treatment options for GCC. Perspectives for future research are discussed. The tumor likely arises from pluripotent intestinal epithelial crypt base stem cells. Previous findings of Notch signaling as a tumor suppressor in Neuroendocrine tumors may have a similar role in this tumor too. 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. A multidisciplinary approach is suggested for optimal outcomes. Surgery remains the main treatment modality. Simple appendectomy may be sufficient in early stages while right hemicolectomy is recommended for advanced tumors. Cytoreductive surgery with heated intraperitoneal chemotherapy may improve survival in a select few with metastatic peritoneal disease. These tumors have an unpredictable behavior even in early stages and local recurrence and delayed metastases may be seen. Lifelong surveillance is warranted.

Keywords: Goblet cell carcinoid of the appendix; Notch-1 signaling; Immunomarkers; Math-1 signaling; Intestinal stem cells; Disease management

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.