Published online May 15, 2024. doi: 10.4251/wjgo.v16.i5.2141
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
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.
To provide an updated overview of risk factors, prognosis, and management of CSRCC.
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.
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.
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.
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.