Systematic Reviews
Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Oncol. May 15, 2024; 16(5): 2141-2158
Published online May 15, 2024. doi: 10.4251/wjgo.v16.i5.2141
Systematic review of risk factors, prognosis, and management of colorectal signet-ring cell carcinoma
Frederiek Nuytens, Vincent Drubay, Clarisse Eveno, Florence Renaud, Guillaume Piessen
Frederiek Nuytens, Clarisse Eveno, Guillaume Piessen, Department of Digestive and Oncological Surgery, University Lille, Claude Huriez University Hospital, Lille 59000, France
Frederiek Nuytens, Department of Digestive and Hepatobiliary/Pancreatic Surgery, AZ Groeninge, Kortrijk 8500, Belgium
Vincent Drubay, Cambrai Hospital Center and Sainte Marie, Group of Hospitals of The Catholic Institute of Lille, Cambrai 59400, France
Clarisse Eveno, Florence Renaud, Guillaume Piessen, CNRS, Inserm, UMR9020-U1277-CANTHER-Cancer, University Lille, CHU Lille, Lille 59000, France
Author contributions: Nuytens F and Drubay V were involved in analysis and interpretation of the data, and drafting and final approval of the manuscript; Eveno C and Renaud F were involved in analysis and interpretation of the data, and revision and final approval of the manuscript; Piessen G was involved in design of the study, decision of publishing the paper, analysis and interpretation of the data, and revision and final approval of the manuscript.
Conflict-of-interest statement: The authors have no potential and real conflicts of interest to disclose.
PRISMA 2009 Checklist statement: The authors have read the PRISMA 2009 Checklist, and the manuscript was prepared and revised according to the PRISMA 2009 Checklist.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Frederiek Nuytens, MD, Surgeon, Department of Digestive and Oncological Surgery, University Lille, Claude Huriez University Hospital, Rue Michel Polonowski, Lille 59000, France. frederiek.nuytens@gmail.com
Received: December 24, 2023
Peer-review started: December 24, 2023
First decision: January 10, 2024
Revised: January 27, 2024
Accepted: March 19, 2024
Article in press: March 19, 2024
Published online: May 15, 2024
Processing time: 137 Days and 3 Hours
Abstract
BACKGROUND

Colorectal signet-ring cell carcinoma (CSRCC) is a rare clinical entity which accounts for approximately 1% of all colorectal cancers. Although multiple studies concerning this specific topic have been published in the past decades, the pathogenesis, associated risk factors, and potential implications on treatment are still poorly understood. Besides the low incidence, historically confusing histological criteria have resulted in confusing data. Nevertheless, the rising incidence of CSRCC along with relatively young age at presentation and associated dismal prognosis, highlight the actual interest to synthesize the known literature regarding CSRCC.

AIM

To provide an updated overview of risk factors, prognosis, and management of CSRCC.

METHODS

A literature search in the MEDLINE/PubMed database was conducted with the following search terms used: ‘Signet ring cell carcinoma’ and ‘colorectal’. Studies in English language, published after January 1980, were included. Studies included in the qualitative synthesis were evaluated for content concerning epidemiology, risk factors, and clinical, diagnostic, histological, and molecular features, as well as metastatic pattern and therapeutic management. If possible, presented data was extracted in order to present a more detailed overview of the literature.

RESULTS

In total, 67 articles were included for qualitative analysis, of which 54 were eligible for detailed data extraction. CSRCC has a reported incidence between 0.1%-2.4% and frequently presents with advanced disease stage at the time of diagnosis. CSRCC is associated with an impaired overall survival (5-year OS: 0%-46%) and a worse stage-corrected outcome compared to mucinous and not otherwise specified adenocarcinoma. The systematic use of exploratory laparoscopy to determine the presence of peritoneal metastases has been advised. Surgery is the mainstay of treatment, although the rates of curative resection in CSRCC (21%-82%) are lower compared to those in other histological types. In case of peritoneal metastasis, cytoreductive surgery with hyperthermic intraperitoneal chemotherapy should only be proposed in selected patients.

CONCLUSION

CSRCC is a rare clinical entity most often characterized by young age and advanced disease at presentation. As such, diagnostic modalities and therapeutic approach should be tailored accordingly.

Keywords: Colorectal cancer; Signet-ring cell histology; Poorly cohesive cells; Systematic review; Risk factors; Prognosis; Therapeutic management

Core Tip: Although multiple studies concerning colorectal signet-ring cell carcinoma (CSRCC) have been published in the past decades, the pathogenesis, associated risk factors, and potential implications on treatment are still poorly understood. As such, the aim of this systematic review was to provide an updated overview of CSRCC by means of a synthesis of the current literature concerning this topic with emphasis on risk factors and clinical and histopathological features of the disease along with diagnostic and therapeutic features that should be tailored to this specific clinical entity.