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Nuytens F, Drubay V, Eveno C, Renaud F, Piessen G. Systematic review of risk factors, prognosis, and management of colorectal signet-ring cell carcinoma. World J Gastrointest Oncol 2024; 16:2141-2158. [PMID: 38764832 PMCID: PMC11099453 DOI: 10.4251/wjgo.v16.i5.2141] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/24/2023] [Revised: 01/27/2024] [Accepted: 03/19/2024] [Indexed: 05/09/2024] Open
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.
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Affiliation(s)
- Frederiek Nuytens
- Department of Digestive and Oncological Surgery, University Lille, Claude Huriez University Hospital, Lille 59000, France
- 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
- Department of Digestive and Oncological Surgery, University Lille, Claude Huriez University Hospital, Lille 59000, France
- CNRS, Inserm, UMR9020-U1277-CANTHER-Cancer, University Lille, CHU Lille, Lille 59000, France
| | - Florence Renaud
- CNRS, Inserm, UMR9020-U1277-CANTHER-Cancer, University Lille, CHU Lille, Lille 59000, France
| | - Guillaume Piessen
- Department of Digestive and Oncological Surgery, University Lille, Claude Huriez University Hospital, Lille 59000, France
- CNRS, Inserm, UMR9020-U1277-CANTHER-Cancer, University Lille, CHU Lille, Lille 59000, France
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Suthar M, Baheti AD, Ankathi SK, Choudhari A, Haria PD, Engineer R, Ostwal V, Ramadwar MS, Desouza A, Saklani A. MRI features of signet ring rectal cancer. Abdom Radiol (NY) 2021; 46:5536-5549. [PMID: 34427742 DOI: 10.1007/s00261-021-03250-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2021] [Revised: 08/10/2021] [Accepted: 08/11/2021] [Indexed: 12/28/2022]
Abstract
PURPOSE Signet Ring Rectal Cancer (SRRC) of rectum is rare high-grade subtype with poor prognosis and characteristic histopathology. We evaluated its imaging appearance and correlated its outcomes. MATERIALS AND METHODS We conducted a retrospective review of the rectal MRIs of 97 patients with rectal SRRC, evaluating tumor morphology, T2 signal, length, location, pattern of tumor growth, nodal status and location, EMVI (extramural vascular invasion), site of metastases, and response to chemotherapy. The tumor signal on T2W images was categorized into intermediate, T2 hyperintense, and fluid/mucin bright. Imaging findings were correlated with risk of metastatic/ recurrent disease, disease-free survival, and overall survival. RESULTS The median age of patients of SRRC in our study was 35 years and more frequently found in male patients. The common imaging features of SRRC were T2-hyperintense signal (63%), infiltrative growth pattern (76%), positive MR CRM (Circumferential Resection Margin on MRI) (84%), presence of EMVI (51%), and advanced T and N stage (97% and 84%, respectively). Peritoneum and nodes were the most common sites of metastases. Raised serum CEA (Carcino-embryonic Antigen) levels, positive MR CRM status, extramesorectal adenopathy, and advanced N stage had statistically significant predictive value for recurrence or metastases. Elevated serum CEA levels (p = 0.019) and intermediate T2 signal (p = 0.012) demonstrated significant independent association with poor overall survival, while advanced N stage (p = 0.033) demonstrated significant independent association with worse disease-free survival in multivariate analysis. CONCLUSION SRRC affected young patients and demonstrated T2-hyperintense signal and subepithelial spread in an infiltrative pattern. Elevated CEA levels and T2-intermediate signal intensity are independent predictors for worse overall survival and advanced nodal stage is independent prognostic factor of poor disease-free survival. MRI rectum can pinpoint the pathology given the distinct MRI morphology and age of presentation.
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Affiliation(s)
- Meena Suthar
- Department of Radio-Diagnosis, Tata Memorial Centre, Mumbai, India
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
| | - Akshay D Baheti
- Department of Radio-Diagnosis, Tata Memorial Centre, Mumbai, India.
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India.
| | - Suman K Ankathi
- Department of Radio-Diagnosis, Tata Memorial Centre, Mumbai, India
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
| | - Amit Choudhari
- Department of Radio-Diagnosis, Tata Memorial Centre, Mumbai, India
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
| | - Purvi D Haria
- Department of Radio-Diagnosis, Tata Memorial Centre, Mumbai, India
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
| | - Reena Engineer
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
- Department of Radiation Oncology, Tata Memorial Centre, Mumbai, India
| | - Vikas Ostwal
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
- Department of Medical Oncology, Tata Memorial Centre, Mumbai, India
| | - Mukta S Ramadwar
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
- Department of Pathology, Tata Memorial Centre, Mumbai, India
| | - Ashwin Desouza
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
- Department of GI Surgical Oncology, Tata Memorial Centre, Mumbai, India
| | - Avanish Saklani
- Tata Memorial Hospital, Homi Bhabha National Institute, Dr. Ernest Borges Road, Mumbai, Maharashtra, 400012, India
- Department of GI Surgical Oncology, Tata Memorial Centre, Mumbai, India
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Abstract
Rectal adenocarcinoma with mucinous components is an uncommon type of rectal cancer with two distinct histologic subtypes: mucinous adenocarcinoma and signet-ring cell carcinoma. Mucin can also be identified as pattern of response after neoadjuvant treatment. On imaging modalities, mucin typically demonstrates high signal intensity on T2-weighted images, low attenuation on computed tomography, and may be negative on 18-fluorodeoxyglucose positron emission tomography. After neoadjuvant CRT, cellular and acellular mucin share similar imaging features, and differentiating them is currently the main challenge faced by radiologists. Radiologists should be aware of pros, cons, and limitations of each imaging modality in the primary staging and restaging to avoid misinterpretation of the radiological findings.
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Horvat N, Hope TA, Pickhardt PJ, Petkovska I. Mucinous rectal cancer: concepts and imaging challenges. Abdom Radiol (NY) 2019; 44:3569-3580. [PMID: 30993392 DOI: 10.1007/s00261-019-02019-x] [Citation(s) in RCA: 39] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
Rectal adenocarcinoma with mucinous components is an uncommon type of rectal cancer with two distinct histologic subtypes: mucinous adenocarcinoma and signet-ring cell carcinoma. Mucin can also be identified as pattern of response after neoadjuvant treatment. On imaging modalities, mucin typically demonstrates high signal intensity on T2-weighted images, low attenuation on computed tomography, and may be negative on 18-fluorodeoxyglucose positron emission tomography. After neoadjuvant CRT, cellular and acellular mucin share similar imaging features, and differentiating them is currently the main challenge faced by radiologists. Radiologists should be aware of pros, cons, and limitations of each imaging modality in the primary staging and restaging to avoid misinterpretation of the radiological findings.
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Affiliation(s)
- Natally Horvat
- Department of Radiology, Hospital Sirio-Libanes, São Paulo, SP, Brazil
| | - Thomas A Hope
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, CA, USA
| | - Perry J Pickhardt
- Department of Radiology, University of Wisconsin School of Medicine and Public Health, Madison, WI, USA
| | - Iva Petkovska
- Department of Radiology, Memorial Sloan Kettering Cancer Center, 1275 York Ave, Box 29, New York, NY, 10065, USA.
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