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 with, however, a rising incidence with relatively young age at presentation and associated dismal prognosis.
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. This highlights the actual interest to synthesize the known literature regarding CSRCC.
The aim of this systematic review was 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.
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. Surgery is the mainstay of treatment, although the rates of curative resection in CSRCC (21%-82%) are lower compared to those of other histological types. In case of peritoneal metastasis, cytoreductive surgery with hyperthermic intraperitoneal chemotherapy should only be proposed in selected patients.
The incidence of CSRCC has risen in the past decades, although it remains a rare histological subtype of CRC. Patients with CSRCC more frequently present at a younger age, with tumors predominantly located in the right colon and rectum. CSRCC is often characterized by locally advanced or even metastatic disease at the time of diagnosis. CSRCC predominantly metastasizes into the peritoneal cavity. As such, oncological outcomes for CSRCC are very poor with a 5-year OS between 0% and 46%.
In light of its aggressive biological behavior and metastatic pattern, a more frequent follow-up after surgical therapy is warranted, with the focus on early detection of peritoneal recurrent disease. The role of immunological therapeutic agents in the case of MSI-H CSRCC remains unclear but will be a topic in future studies.