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Background - Objective:  Primary colorectal squamous cell carcinoma is an extremely rare neoplasm with an incidence of 0.1 to 0.25 per 1,000 diagnosed colorectal carcinomas.The objective of this study was to evaluate its biological behavior and highlight the role of a surgical approach for its management. Methods PubMed and Cohrane databases were independently searched (last search: April 10th, 2020) for articles concerning colorectal squamous cell carcinoma in adult population. Results:  Seventy-one studies met predefined inclusion criteria and involved 99 patients (54.5% females) with an age of 56.98 ± 12.19 years (mean ± SD). The most frequent site of occurrence was the rectum (63.5%). Open surgery was conducted at 95% of patients, while 21.4% and 30.3% received neoadjuvant and adjuvant therapy respectively.Postoperative complications were developed in 31.3% of patients, while 6.1% died withing the first month following operation. Five-years survival rate was 49.5% (95% CI: 33.7%-63.4%). Female sex (HR: 0.24; 95% CI: 0.11–0.54; p-value: 0.001) and presence of postoperative complications (HR: 4.10; 95%CI: 1.47–1.46; p-value: 0.007) significantly affected the survival. Conclusions Colorectal Squamous Cell Carcinoma is a rare tumor with an aggressive behavior. Surgery is the standard of treatment for the colontumors, while the role of chemoradiotherapy is promising especially for rectal tumors. Further clinical trials are necessary to determine the preferred treatment approach.

Primary Squamous Cell Carcinoma of Colon and Rectum: A Systematic Review of the Literature